Abbreviations:

a       = alpha
B       = beta
r       = gamma
k       = kappa
ACTH    = adrenocorticotropic hormone
CSF     = cerebrospinal fluid
DEC     = states with decreased levels
DIC     = disseminated intravascular coagulopathy
FP      = function, properties, genetic variants
GV      = genetic variant
HDL     = high-density lipoprotein
HLA     = human leukocyte antigen
HS      = heat soluble
IDL     = intermediate-density lipoprotein
INC     = states with increased levels
J       = joining piece
LCAT    = lecithin cholesterol acyl transferase
LDL     = low-dendity lipoprotein
LMW     = low molecular weight
MO      = electrophoretic mobility (agarose pH 8.6)
MW      = molecular weight (daltons)
PMNL    = polymorphonuclear leukocyte
RI      = reference interval (g/l [IU/ml])
SC      = secretory component
TIBC    = total iron-binding capacity
VLDL    = very-low-density lipoprotein



********************************************************************************
                                 IMMUNOGLOBULINS
********************************************************************************
Immunoglobulin G (7s-r-globulin) (IgG); MW: 150,000; MO: a2 - r2
RI: 8.0 - 17.0 [92 - 196]
FP: Antibodies, esp. to viruses, bacteria, toxins
    Only Ig which crosses the placenta (active transport)
    Subclasses: IgG1, IgG2, IgG3, IgG4
    GV: GM allotypes of r chains, KM allotypes of k chains
INC Chronic liver disease (incl. cirrhosis)
    Chronic infection
    Parasitic disease
    Sarcoidosis
    IgG myeloma
DEC Acquired immunodeficiency
    Hereditary deficiencies (class or subclass)
    Protein-losing syndromes
    Pregnancy
    Non-IgG myeloma
    Waldenstrom's macroglobulinemia
================================================================================
Immunoglobulin A (IgA); MW: 160,000 and aggregates; MO: B2 - r1
RI: M: 1.0 - 4.9 [60 - 292] F: 0.85 - 4.5 [51 - 268]
FP: Antibodies subclasses: IgA1, IgA2
    GV: AM allotypes of a chains, KM allotypes of k chains
INC Chronic liver disease (incl. cirrhosis)
    Chronic infection, esp. GI and resp. tracts
    Inflammatory bowel disease
    Neoplasia of GI tract
    Autoimmune disease. esp. rheumatoid arthritis
    Wiskott-Aldrich syndrome
    IgA myeloma (often polymerizing)
DEC Acquired immunodeficiency
    Hereditary deficiencies (class or subclass)
    Ataxia telangiectasia
    Protein-losing syndromes
    Infancy, early childhood
    Non-IgA myeloma
    Waldenstrom's macroglobulinemia
================================================================================
Secretory IgA (s IgA); MW: 385,000; MO: r1
FP: Antibodies, esp. in mucous secretions and colostrum [2 IgA + secretory
    component (SC) + joining (J) chain]
DEC Secretory IgA deficiency
================================================================================
Immunoglobulin M (19s-r-globulin) (IgM); MW: 971,000 (pentamers); MO: r1 - r2
RI: M: 0.5 - 3.2 [58 - 368] F: 0.6 - 3.7 [69 - 425]
FP: First antibodies to appear after antigenic stimulus
    Isohemagglutinins (Anti-A, -B)
    May be present as monomers, esp. in infancy or with plasma cell dyscrasia
INC Acute/chronic infection, esp. viral
    Waldenstrom's macroglobulinemia
    Nephrotic syndrome
    Autoimmune disease/Sarcoidosis
    Hepatocellular disease
    Estrogen/pregnancy
    Hyper-IgM dysgammaglobulinemia
    Congenital infection (newborn infants)
DEC Acquired immunodeficiency
    Hereditary deficiencies
    Protein-losing syndromes
    Infancy, early childhood
    Non-IgM myeloma
================================================================================
Immunoglobulin D (IgD); MW: 175,000; MO: r1
RI: < 0.15 [< 100]
FP: Antibodies
    Antigen receptor on B lymphocytes
INC Autoimmune disease
    Chronic infections
    IgD myeloma
DEC Hereditary deficiencies/ Infancy
    Acquired Immunodeficiency
    Non-IgD myeloma
================================================================================
Immunoglobulin E (IgE); MW: 190,000; MO: r1
RI: < 0.0003 [< 100]
FP: Antibodies (reagins)
INC Allergies
    Parasitic disease
    IgE myeloma
DEC Hereditary deficiencies
    Acquired immunodeficiency
    Ataxia telangiectasia
    Non-IgE myeloma




********************************************************************************
                                    INHIBITORS
********************************************************************************
a1-Antitrypsin (a1-Proteinase inhibitor) (a1 Pl, a1 A); MW: 54,000; MO: a1
RI: 1.4 - 3.2 [59 - 134]
FP: Inhibits serine proteinases esp. PMNL elastase
    GV: PI types; over 75 known, many hypomorphic (Z, S, P, Null, etc.)
INC Estrogen/pregnancy
    Anabolic steroids
    Acute phase response
    Hepatocellular disease (acute or chronic)
DEC Nephrotic syndrome
    Hereditary deficiency (emphysema, hepatoma; neonatal cholestasis, infantile
    cirrhosis)
    Terminal liver disease/Severe hepatitis
    Respiratory distress syndrome of infancy
    Acute pancreatitis
================================================================================
a1-Antichymotrypsin (a1X-Glycoprotein)(a1 X); MW: 68,000; MO: Slow a1 - Inter-a
RI: 0.3 - 0.6
FP: Inhibits cathepsin G. mast cell chymase
INC Acute phase response
DEC Hereditary deficiency/Infancy
    Nephrotic syndrome
    Status asthmaticus
================================================================================
Inter-a-Trypsin inhibitor (IaTI); MW: 160,000; MO: Inter-a
RI: 0.2 - 0.7
FP: Inhibits trypsin, acrosin, secretory proteinases
    Cleaced by PMNL elastase to 2 fragments; small fragment is a high-affinity
    inhibitor of most serine proteinases
    Precursor of LMW inhibitor in secretions and tissues ?
INC Inflammatory disorders
    Malignancy
================================================================================
Antithrombin III (heparin cofactor) (AT III); MW: 58,000; MO: Inter-a to a2
RI: 0.22 - 0.39 (plasma)
FP: Inhibits thrombin, factors Xa, XII, XI, IX; kallikrein
    Activity greatly potentiated by heparin
    GV: qualitative and quantitative
INC Acute phase response
    Anabolic steroids
DEC Nephrotic syndrome
    Hereditary deficiency (hypercoagulability)/Oral contraceptives
    Disseminated intravascular coagulopathy (DIC)/Deep vein thrombosis/Lung
    embolism
================================================================================
a-Thiol Proteinase Inhibitor (LMW kininogen) (a TPI); MW: 167,000 90,000
MO: a1 a2
RI: 0.3 - 0.5
FP: Inhibits thiol proteinases (cathepsins B1, C, H, L, and Ca++ -activated
    neutral protease)
DEC Infancy
================================================================================
C1-Inactivator (C1 esterase inhibitor) (a2-neuraminoglycoprotein) (C1 INA)
MW: 104,000; MO: a2
RI: 0.15 - 0.35
FP: Inhibits activated complement components C1r, C1s; clotting and fibrinolytic
    factors; kallikrein, plasmin
    GV: quantitative and qualitative variants (some hypofunctional)
INC Acute phase response
DEC Hereditary deficiency (hereditary angioedema)
================================================================================
a2-Macroglobulin (a2 M); MW: 725,000; MO: a2
RI: M: 1.2 - 2.7 [50 - 113] F: 1.4 - 3.2 [59 - 134]
FP: Inhibits most (? all) endoproteinases, including clotting, fibrinolytic, &
    complement factors
    May bind/transport Zn++
    GV: Xm system
INC Nephrotic syndrome
    Liver disease
    Diabetes mellitus
    Childhood
    Estrogen/pregnancy
DEC Fibrinolysis; DIC
    Acute pancreatitis
    Stress
    Severe peptic ulcer disease
    Extracorporeal circulation
    (No hereditary deficiency)
================================================================================
a2-Antiplasmin (a2 AP); MW: 65,000; MO: a2
RI: 0.04 - 0.08
FP: Inhibits plasmin (fibrinolysin), kallikrein
INC Acute phase response
    Diabetes mellitus
DEC Hereditary deficiency (hyperfibrinolysis)
================================================================================
Cystatin C (Post-r-globulin) (r-Trace protein); MW: 11,500; MO: Post-r
RI: Trace
FP: Inhibits cysteine
    Proteinases
INC In CSF: demyelinating disorders
    In urine: tubular damage



********************************************************************************
                             COMPLEMENT COMPONENTS
********************************************************************************
C1q (11S protein); MW: 410,000; MO: r2
RI: 0.05 - 0.25
FP: Recognition unit for complement activation via classical pathway; binds
    immune aggregates with IgM, some IgGs via 6 globular "heads"
    Sequence similarity to collagen
INC Acute phase response
DEC Lupus erythematosus
    Lymphopenia
    Hereditary deficiency
    Infancy
================================================================================
C1r ; MW: 166,000 (dimer); MO: B
RI: 0.025 - 0.1
FP: Proenzyme activated by complexed C1q; activates C1s
    GV
DEC Lupus erythematosus
    Renal disease/Recurrent infections
    Hereditary deficiency/Infancy
================================================================================
C1s ; MW: 83,000; MO: a2
RI: 0.05 - 0.1
FP: Proenzyme; binds C1q and C1r via Ca++; activates C4 and C2
    GV
INC Acute phase response
DEC Lupus erythematosus
    Infancy
================================================================================
C2 ; MW: 102,000; MO: B1
RI: 0.01 - 0.08
FP: Proenzyme, activated by C1, with release of kinin-like fragment; part of
    C3 and C5 convertases
    GV: linked to HLA, C2, factor B
INC Acute phase response
DEC Immune complex disease, esp. lupus erythematosus with nephritis
    Hereditary angioedema (HANE)
    Hereditary deficiency/Infancy
================================================================================
C3 (B1C-globulin); MW: 185,000; MO: B1 - B2
RI: 0.5 - 0.9 [0.64 - 1.15]
FP: C3a: cnaphylatoxin
    C3b: opsonization, chemotaxis; part of C5 convertase (and C3 convertase of
    altervative pathway)
    GV: some hypomorphic
INC Subacute inflammation
    Biliary obstruction
    Nephrotic syndrome (+)
    Corticosteroid therapy (+)
DEC Immune complex disease, esp. lupus erythematosus with nephritis
    Acute poststreptococcal glomerulonephritis
    Hypercatabolism, esp. C3b inactivator deficiency
    Hereditary deficiency (recurrent infections)/infancy
================================================================================
C4 (B1E-globulin); MW: 200,000; MO: B1
RI: 0.1 - 0.4
FP: C4a: anaphylatoxin
    C4b: constituent of C3 & C5 convertases in the classical pathway
    GV: two loci (C4A, C4B); linked to HLA, C2, factor B
INC Acute phase response
    Estrogen/pregnancy
DEC Immune complex disease, esp. lupus erythematosus with nephritis
    Hereditary angioedema (HANE)
    Hereditary Deficiency
    Acute glomerulonephritis/Infancy
================================================================================
C5 (B1F-globulin); MW: 185,000; MO: B1
RI: 0.04 - 0.15
FP: C5a: anaphylatoxin, chemotaxis
    C5b: constituent of membrane attack complex (C5b-C9)
    GV (familial dysfunction)
DEC Leiner's disease (functional deficiency)
    Lupus erythematosus
    Hereditary deficiency (recurrent neisserial infections)
================================================================================
C6 ; MW: 104,800; MO: B2; RI: 0.04 - 0.08
C7 ; MW:  92,400; MO: B2; RI: 0.05 - 0.08
C8 ; MW: 163,000; MO: r1; RI: 0.04 - 0.08
C9 ; MW:  71,000; MO: a;  RI: 0.05 - 0.25
FP: Constituents of membrane attack complex
    GV
DEC Hereditary deficiency (recurrent neisserial infections)
================================================================================
Factor B (C3-proactivator; B2-glycoprotein II; glycine-rich B-glycoprotein)
MW: 90,000; MO: B1; RI: 0.1 - 0.4
FP: Proenzyme precursor of C3 activator in alternative pathway
    GV (linked to C2, C4, HLA)
INC Acute phase response
DEC Lupus erythematosus, other immune complex diseases
    Infections with gram-negative organisms
    Sickle cell disease
================================================================================
Factor D (C3-proactivator convertase); MW: 24,000; MO: a; RI: approx. 0.01
FP: Proenzyme, activation of factor B in alternative pathway
================================================================================
Properdin (P); MW: 220,000 (tetramer); RI: 0.01 - 0.03
FP: Regulator in alternative pathway (stabilizes C3bBb)
DEC Acute glomerulonephritis
    Gram-negative sepsis
================================================================================
Factor I (C3b inactivator); MW: 90,000; MO: B; RI: 0.025 - 0.05
FP: Proteolytic cleavage of C3b, C4b, C5b
DEC Hereditary deficiency (recurrent infections)
================================================================================
C4-binding Protein; MW: 540,000; MO: B2; RI: 0.18 - 0.32
FP: Enhancement of C4b cleavage (with C3bINA)



********************************************************************************
                         COAGULATION and FIBRINOLYSIS
********************************************************************************
Fibrinogen (F I); MW: 340,000; MO: B2; RI: 2.0 - 4.0 (plasma)
FP: Precursor of fibrin, the major constituent of blood clots
    GV: qualitative and quantitative (some hypofunctional)
INC Acute phase response
    Estrogens, pregnancy, oral contraceptives
DEC DIC / Hepatocellular disease
    Anabolic steroids
    Hereditary deficiency/Infancy
================================================================================
Prothrombin (F II); MW: 72,000; MO: a1; RI: 0.05 - 0.1 [0.5 - 1.5 kU/L)
FP: Proenzyme of thrombin, which converts fibrinogen to fibrin
    Vitamin K-dependent; GV
INC Estrogens, oral contraceptives
DEC Hepatocellular disease
    Vitamin K deficiency / Oral anticoagulants
    Hereditary deficiency
================================================================================
Factor V (proaccelerin) (F V); MW: 330,000; MO: B; RI: < 0.03 [0.5 - 2 kU/L]
FP: Accessory factory (nonenzymatic) in the prothrombinase complex (with tissue
    factor)
DEC Hepatocellular disease
    Septicemia, DIC
    Acquired inhibitors
    Hereditary deficiency
================================================================================
Factor VII (proconvertin) (F VII); MW: 48,000; MO: a; RI: < 0.001
FP: Coactivator (with tissue factor) of extrinsic coagulation pathway
    Vitamin K-dependent
INC Estrogens, oral contraceptives
    Anabolic steroids
DEC Hepatocellular disease
    Vitamin K deficiency / Oral anticoagulants
    Aspirin, dextrothyroxine
    Hereditary deficiency
================================================================================
Factor VIII:C(antihemophilic factor)(F VIII:C); MW: 92,000 & 80,000 (two chains)
MO: B; RI: < 0.001
FP: Pro-coagulant protein
    Constituent of factor X activator complex
INC Acute phase response
    Exercise
    Late pregnancy
    Uremia
DEC Acquired inhibitors
    DIC (up or down)
    Hereditary deficiency; hemophilia A (X-linked)
================================================================================
Factor VIII-Related Antigen (F VIII:RAg) (von Willebrand factor) (VWF)
MW: 1,000,000; MO: B; RI: 0.005 - 0.01
FP: Normally complexed with F VIII procoagulant
    Essential for platelet aggregation
INC Acute phase response
    Exercise
    Uremia
DEC Hereditary deficiency (von Willebrand's disease), autosomal dominant
================================================================================
Factor IX (Christmas factor) (F IX); MW: 51,800; MO: a1; RI: 0.005 - 0.01
FP: Proenzyme, constituent of factor X activation complex
    Vitamin K-dependent
    GV
INC Estrogens
    Glucocorticosteroids
DEC Hepatocellular disease
    Vitamin K deficiency / oral anticoagulants
    Hereditary deficiency: hemophilia B (Christmas disease) (X-linked)
================================================================================
Factor X (Stuart-Prower factor) (F X); MW: 59,000; MO: a1; RI: 0.005 - 0.01
FP: Proenzyme, constituent of prothrombin activator complex
    Vitamin-K dependent; GV
INC Anabolic steroids
    Estrogens
DEC Hepatocellular disease / Amyloidosis
    Vitamin K deficiency / Oral anticoagulants
    Hereditary deficiency
================================================================================
Factor XI (plasma thromboplastin antecedant) (F XI); MW: 130,000
RI: 0.004 - 0.006
FP: Proenzyme, activated by factor XIIa; activates factor IX
DEC Hereditary deficiency; hemophilia C
    DIC
================================================================================
Factor XII (Hageman factor) ("glass factor" - ed.) (F XII); MW: 76,000
MO: a; RI: 0.015 - 0.05
FP: Proenzyme, activated by surface contact; activates factor XI, with HMW
    kininogen
    F XIIa also activates complement, kallikrein, fibrinolytic systems
INC Oral contraceptives
DEC Hereditary deficiency
    DIC
================================================================================
Factor XIII (fibrin stabilizing factor) (F XIII); MW: 320,000; MO: a
RI: 0.01 - 0.04
FP: Transamidase; cross-linking of fibrin monomers to stabilize blood clots
DEC Hepatocellular disease
    Hereditary deficiency
    DIC
================================================================================
High-Molecular-Weight (HMW) Kininogen (Fitzgerald factor); MW: 197,000
MO: a; RI: 0.09 +/-
FP: Accessory in contact-phase activation
DEC Chronic renal failure
    Hepatic cirrhosis
    DIC
================================================================================
Prekallikrein (Fletcher factor); MW: 95,000; MO: a; RI: 0.09 - 0.11
FP: Proenzyme; enzymatic and accessory function in contact-phase activation
DEC Severe liver disease
    Fibrinolysis, DIC/Uremia
    Hereditary deficiency/Infancy
================================================================================
Plasminogen ; MW: 91,000; MO: B; RI: 0.06 - 0.25 (plasma)
FP: Proenzyme of plasmin (fibrinolysin), which lyses fibrin clots
    Activated by tissue plasminogen activators (tPA), streptokinase, urokinase
    Affinity binding to fibrin (?)
    GV: some hypofunctional
INC Anabolic steroids
    Estrogens/pregnancy
    Rigorous exercise
DEC Fibrinolytic therapy
    Severe liver disease
    DIC
    Fetus/Infancy
    Infant respiratory distress syndrome
================================================================================
Protein C; MW: 62,000; RI: approx. 0.001
FP: Proenzyme "anticoagulant"; inactivates factors Va and VIIIa by limited
    proteolysis; vitamin K-dependent
DEC Hereditary deficiency
    Vitamin K deficiency
    Oral anticoagulants
================================================================================
Protein S; MW: 69,000; RI: Trace
FP: Enhances activity of protein C
    Two forms in plasma, free and complexed to C4 binding protein
    Vitamin K-dependent
DEC Hereditary deficiency
    Vitamin K deficiency
    Oral anticoagulants



********************************************************************************
                               BINDING / TRANSPORT
********************************************************************************
Albumin (ALB); MW: 66,460; MO: Albumin; RI: 37 - 53 [93 - 133]
FP: Maintenance of oncotic osmotic pressure
    Transport of fatty acids, bilirubin, metal ions
    GV of albumin and proalbumin
INC Dehydration
    Intravenous infusion
DEC Acute phase response
    Chronic inflammation
    Malnutrition/Protein-losing syndromes
    Fluid shifts/Overhydration
    Hereditary deficiency
================================================================================
Haptoglobin (HP); MO: a2; RI: 0.5 - 3.2
Hp 1-1: MW: 86,018 RI: 1.0 - 2.3
Hp 2-1: MW: 86,018+ polymers RI: 0.9 - 3.2
Hp 2-2: MW: > 200,000 (polymers) RI: 0.5 - 3.2
FP: Binds free hemoglobin, resulting in: peroxidase activity and preservation
    of body iron
    Tetrameric (2a, 2B chains); Hp 2 forms polymers
    GV: qualitative, quantitative
INC Acute phase response
    Glucocorticoids
    Hodgkin's disease
    Anabolic steroids
    Biliary obstruction
    Nephrotic syndrome (Hp 2-1 or 2-2)
    Ulcerative colitis
DEC In vivo hemolysis or ineffective erythropoiesis
    Estrogen/Pregnancy
    Active hepatocellular disease
    Nephrotic syndrome (Hp 1-1)
    Infancy
================================================================================
Prealbumin (transthyretin, thyroxine-binding prealbumin); MW: 54,980 (tetramer)
MO: Prealbumin; RI: 0.25 - 0.45
FP: Binds and transports T3/T4 & retinol-binding protein
    GV: some ? associated with familial amyloidosis
INC Glucocorticosteroids
    Hodgkin's disease
    Nonsteroidal anti-inflammitory therapy
    Alcoholism (without hepatocellular disease)
DEC Acute phase response
    Hepatocellular disease
    Malnutrition
    Nephrotic syndrome
    Infancy, early childhood
    Estrogens/Pregnancy
================================================================================
Retinol-binding Protein (RBP); MW: 20,960; MO: a2 alone; Prealbumin complexed
RI: 0.03 - 0.06
FP: Binds and transports retinol (vitamin A); essential for cellular
    recognition and uptake of vitamin A
    Complexed 1:1 with prealbumin; concentrations correlate except with
    vitamin A deficiency
INC Glucocorticosteroids
    Oral contraceptives
    Renal failure
DEC Acute phase response
    Hepatocellular disease
    Malnutrition
    Nephrotic syndrome
    Hyperthyroidism
    Vitamin A deficiency
================================================================================
Thyroxine-binding Globulin (TBG); MW: 54,000 MO: a1 to Inter-a
RI: 0.01 - 0.03
FP: Binds and transports T3, T4
    GV: qualitative and quantitative (X-linked)
INC Estrogen/Pregnancy
    Hereditary excess
    Hepatitis B
    Hypothyroidism (+/-)
DEC Acute phase response
    Anabolic steroids
    Glucocorticoids
    Hyperthyroidism
    Malnutrition
    Nephrotic syndrome
    Hereditary deficiency (X-linked)
================================================================================
Transcortin (corticosteroid-binding globulin) (CBG); MW: 55,700; MO: Inter-a
RI: 0.015 - 0.02
FP: Binds and transports cortisol, etc.
INC Estrogen/Pregnancy
DEC Anabolic steroids
    Ovarian hypofunction
================================================================================
Sex hormone-binding Globulin (Steroid-binding B Globulin) (SHBG); MW: 115,000
MO: B1; RI: M: 0.001 - 0.012  F: 0.003 - 0.015
FP: Binds and transports testosterone, estradiol, etc.
INC Estrogen/Pregnancy
DEC Anabolic steroids
    Thyroid hormones
    Groth hormone
================================================================================
Vitamin D-binding Protein (Gc-globulin, group specific component) (VDBP)
MW: 50,800; MO: Inter-a to a2
RI: 0.2 - 0.55
FP: Binds and transports vitamin D3; binds/scavenges actin
    GV: GC (over 50 alleles)
INC Estrogen/Pregnancy
DEC Severe liver disease
    Protein-losing syndromes
    Infancy
================================================================================
Transcobalamin I (TC I); MW: 65,000; MO: a1; RI: Trace
FP: Rapid binder of vitamin B12
INC Hepatoma
    Polycythemia
    Myeloid leukemia
================================================================================
Transcobalamin II (TC II); MW: 53,900; MO: B1; RI: > 25 ng/L
FP: High-turnover vitamin B12 binding and transport
    GV: including hypofunctional
INC Myeloproliferative disorders
DEC Hereditary deficiency
    Infancy
================================================================================
Transferrin (siderophilin) (TF); MW: 79,550; MO: B1
RI: 2.3 - 4.3 [80 - 150]
FP: The major binding/transport protein for iron; 1 mg = 1.25 ug Fe+++ in total
    iron-binding capacity (TIBC)
    Unsaturated transferrin (or low free iron) may be important in control of
    infections, parasitic infestations
    GV: over 20 known
INC Iron deficiency
    Estrogen/Pregnancy
DEC Acute phase response
    Chronic inflammation
    Malnutrition
    Hemochromatosis
    Protein-losing syndromes
    Hereditary deficiency (severe hypochromic anemia)
================================================================================
Ferritin ; MW: 450,000
RI: M: 30 - 220 ug/L  F: 20 - 110 ug/L
FP: Plasma concentration proportional to iron stores except with acute phase
    Primary iron storage protein in most body tissues, esp. liver, spleen, bone
    marrow
INC Iron overload, esp. hemochromatosis and chronic transfusion
    Acute/chronic inflammation
    Active liver disease
DEC Iron deficiency
    Generalized malnutrition
================================================================================
Hemopexin (HPX); MW: 60,000; MO: B1; RI: 0.5 - 1.15
FP: Binds free heme, conserving body iron
INC Estrogen/Pregnancy
    Acute phase response
DEC Severe in vivo hemolysis
    Nephrotic syndrome
    Infancy




********************************************************************************
                                APOLIPOPROTEINS
********************************************************************************
Apolipoprotein A
Apo A-I: MW: 28,020 MO: a1; RI: 1.15 - 2.1
FP: Major protein constituent of high-density lipoprotein (HDL); activates
    lecithin-cholesterol acyl transferase (LCAT) in vitro
    Stabilizes prostacyclin, reduces platelet aggregation
    GV: some malfunctional
INC Estrogen/Pregnancy
    Exercise
    Familial increase
    Liver disease
    (Increase is associated with decreased risk for atherosclerosis)
DEC Familial Hypo-a-lipoproteinemia
    Tangier disease (hereditary)
    "Fish-eye" disease (hereditary)
    Cholestasis; sepsis
    (Decrease is associated with increased risk for atherosclerosis)
Apo A-II: MW: 17,440; MO: a1; RI: 0.26 - 0.66
FP: Structural component of HDL; function otherwise unknown; GV
DEC Cholestasis
================================================================================
Apolipoprotein B
Apo B-48: MW: approx. 250,000; MO: B; RI: (Varies with dietary status)
FP: Major protein of chylomicrons; synthesized by intestine
INC Liver disease
Apo B-100: MW: approx. 514,000; MO: B; RI: 0.65 - 1.55
FP: Primary protein component of low-density lipoproteins (LDL) and very-low-
    density lipoproteins (VLDL)
    Cholesterol transport; ligand for LDL (apo B/E) receptors
    Synthesized primarily in the liver
    GV: at least one qualitative variant is associated with elevated LDL
INC Pregnancy, progestins
    Familial hypercholesterolemia
    Familial hyper apobetalipoproteinemia
    Nephrotic syndrome
    Biliary obstruction
    Type II hyperlipidemia
    (Increase is associated with increased risk for atherosclerosis)
DEC Estrogens
    Familial hypobetalipoproteinemia
    Abetalipoproteinemia
    Sepsis
    Liver disease
    (Decrease is associated with decreased risk for atherosclerosis)
================================================================================
Apolipoprotein C
Apo C-I: MW: 6,630; RI: 0.04 - 0.06
FP: Minor component of most lipoproteins, esp. VLDL, chylomicrons, chylomicron
    remnants
    May activate LCAT
Apo C-II: MW: 8,824; RI: 0.03 - 0.05
FP: Lipoprotein lipase activation (with apolipoprotein H)
    Most found in VLDL, chylomicrons
    GV: qualitative, quantitative (some nonfunctional)
DEC Hereditary deficiency (autosomal recessive; very high triglycerides)
Apo C-III: MW: 8,764; RI: 0.12 - 0.16
FP: Inhibits remnant uptake by interfering with ApoE receptor
    May modulate lipoprotein lipase activity
    GV: one qualitative variant associated with hypertriglyceridemia
DEC Combined hereditary deficiency with ApoA-I (with decreased HDL and increased
    risk for atherosclerosis)
================================================================================
Apolipoprotein E (apo E); MW: 34,145; RI: M: 0.02 - 0.06  F: 0.015 - 0.04
FP: Constituent of chylomicron remnants, VLDL, intermediate-density lipoproteins
    (IDL), LDL, some HDL; ligand for ApoE and B/E receptors (removal of lipids,
    esp. by liver)
    GV: most individuals with type III hyperlipidemia are phenotype E2/2
INC Familial increase
    Pregnancy
    Dexamethasone
    Cholestasis
    In cerebrospinal fluid (CSF): remyelination (e.g., recovery phase of
    multiple sclerosis)
DEC Familial decrease
    Adrenocorticortropic hormone (ACTH)
    Liver disease
================================================================================
Apolipoprotein (a) (apo (a)); MW: approx. 280,000 (varies with phenotype)
MO: Pre-B; RI: 0.01 - 1.4 (varies with phenotype)
FP: Constituent of Lp(a), bound to apo B-100 via disulfide linkage. Atherogenic
    Partial sequence homology with plasminogen; blocks plasminogen binding to
    fibrin clots ?
    GV: qualitative, quantitative
INC Hereditary elevation
DEC Hereditary decrease
    Anabolic steroids
================================================================================
Serum Amyloid A (SAA); MW: 11,685 (100,000 - 180,000 in serum); RI: < 0.001
FP: Present in HDL fraction; function unknown
    Precursor to amyloid a fibrils ?
INC Acute phase response (300 - 500 x increase)
    Pregnancy
    Amyloidosis




********************************************************************************
                                      OTHER
********************************************************************************
a-fetoprotein (AFP); MW: 66,300; MO: a1; RI: < 10 ug/L [<7]
FP: Fetoembryonal analogue of albumin, with similar function thereto;
    senthesized by yolk sac and fetal liver
    (Some fractions may bind estrogens and/or be immunosuppressive)
INC Infancy/Pregnancy
    Hepatocellular cancer, esp. hepatoblastoma
    Choriocarcinoma/teratoma, esp. of testis/ovary
    Acute hepatitis/Hepatic regeneration
    Ataxia telangiectasia
    Hereditary tyrosinemia
    Congenital hypothyroidism
    maternal serum: open fetal defects of neural tubes, abdominal wall; twin
    pregnancy; etc.
DEC In maternal serum: fetal death; trisomy 21
================================================================================
a1-Acid Glycoprotein (orosomucoid) (a1 AG); MW: 41,000
RI: M: 0.50 - 1.30  F: 0.40 - 1.20
FP: Binds (? inactivates) basic hormones, drugs (e.g. progesterone, propanolol)
    Suppresses immunoreactivity
    Modifies platelet adhesiveness
    GV: two loci (ORM1, ORM2)
INC Acute phase response
    Glucocorticoids (endogenous/exogenous)
    Physical exertion
    Renal failure
DEC Estrogen/Pregnancy
    Protein-losing syndromes
    Severe liver disease
    Infancy
================================================================================
Ceruloplasmin (CER); MW: 132,000; MO: Inter-a to a2
RI: 0.20 - 0.55 [64 - 176]
FP: Reduction/oxidation; ferrioxidase; oxygen radical scavenger
    Transports Cu++ (1% by alb) ?
    Very susceptible to proteolytic degradation
    GV
INC Estrogen/Pregnancy
    Acute phase response
    Reticuloendothelial disease (e.g., Hodgkin's disease)
    Biliary obstruction
DEC Hereditary deficiency (iron storage disease)
    Secondary deficiency: Wilson's disease (hepatocellular degeneration);
    Menke's steely (kinky) hair syndrome
    Copper deficiency
    Severe liver disease
    Nephrotic syndrome
    Infancy
================================================================================
Serum Amyloid P Protein (9.5s a1-glycoprotein; a1-macroglobulin) (SAP)
MW: 233,000 - 308,000; MO: a1; RI: 0.035 - 0.075
FP: Constituent of amyloid deposits; lecithin-like properties
    High affinity for polyanions and cations, fibronectin
    Pentagonal (double pentamers); much sequence and structural homology with
    C-reactive protein
DEC Hepatocellular disease
================================================================================
a2-HS Glycoprotein (a2 HS); MW: 50,000; MO: a2; RI: 0.40 - 0.85
FP: Component of bone matrix and dentin; affinity for Ca++, Zn++
    Opsonic properties ?
    Depresses cell-mediated immunity ?
    GV
DEC Acute phase response
================================================================================
Fibronectin (cold insoluble globulin) (FN); MW: 440,000 +/-; MO: a2 - B1
RI: 0.25 - 0.4
FP: Promotes cell-cell adhesion
    Binds to fibrin (clots) and C1q (immune complexes), resulting in
    opsonization and fibroblast adherence
INC Chronic active hepatitis
DEC Acute phase response
    Sepsis; shock; DIC
    Acute leukemia
    Acute pancreatitis
    Hereditary deficiency
================================================================================
C-reactive Protein (CRP); MW: 105,000 - 114,700
MO: B - r2 (Depending on Ca++ concentration); RI: < 0.005
FP: Activates complement (Ca++ dependent), resulting in early, nonimmune
    clearance of gram-negative bacteria and ? tissue breakdown products
    A pentraxin, structurally related to serum amyloid P
    Binds phosphorylcholine in the presence of Ca++
INC Acute phase response (up to 1000 x increase)
DEC Infancy
    (no reported hereditary deficiency)
================================================================================
B2-Microglobulin (B2 M); MW: 11,818; MO: B2; RI: 0.001 - 0.003
FP: Component of human leukocyte antigen (HLA) molecules class I
INC B-cell malignancies
    Renal failure
    Fetal/neonatal period
    In urine: proximal renal tubular disease; renal transplant rejection
================================================================================
Pregnancy-specific B1-glycoprotein (SP1); MW: 90,000 (42,300) n; MO: B1
RI: < 1/1,000,000
FP: Binds and transports steroid hormones
    Synthesized by the syncytiotrophoblast; concentration proportional to
    placental weight
INC Pregnancy
    Trophoblastic disease
    Malignant teratomas
    Twin pregnancy
DEC "Blighted ovum"
    Preceeding spontaneous abortion
    Placental insufficiency
================================================================================
a1-Microglobulin (a1 M); MW: approx. 33,000; MO: a1; RI: 0.02 - 0.05
FP: Immunosuppression
    Binds lipophilic molecules (a lipocalin, related to retinol binding protein
    and a1-acid glycoprotein)
INC Renal insufficiency
    In urine: tubular proteinuria
================================================================================

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