antibody

Anti-Human Factor H (Biotin) (Clone: OX-24) (mouse IgG1)

MBS520287

0.1 mg

245 USD

monoclonal

mouse

biotin

OX-24

ELISA, enzyme immunoassay

Antibody

Anti-Human Factor H (Biotin) (Clone: OX-24) (mouse IgG1)

Factor H

Factor H (Biotin) (Clone: OX-24) (mouse IgG1); Biotin Anti-Human Factor H Monoclonal Antibody

factor H; Complement factor H; complement factor H; beta-1H; factor H-like 1; beta-1-H-globulin; H factor 1 (complement); H factor 2 (complement); adrenomedullin binding protein; complement factor H, isoform b; age-related maculopathy susceptibility 1; complement factor H; H factor 1

CFH; CFH; FH; HF; HF1; HF2; HUS; FHL1; AHUS1; AMBP1; ARMD4; ARMS1; CFHL3; HF; HF1; HF2

CFAH_HUMAN

Monoclonal

Mouse IgG1

OX-24

Mouse

Human

1231

Factor H (Human Factor H)

Biotin

Antibody Concentration: 0.1 mg/ml

Store at 4 degree C. For long term storage, aliquot and freeze unused portion at -20 degree C in volumes appropriate for single usage. Avoid freeze/thaw cycles.

ELISA (EIA)

Immunogen: Human complement Factor H. Donor: BALB/c mouse spleen cells. Fusion Partner: NS-O myeloma

Presentation: 100 ug Biotin conjugated Ig buffered in PBS, 0.02% NaN3 and EIA grade BSA as a stabilizing protein to bring total protein concentration to 4-5 mg/ml.

anti-human factor H (beta 1H) (139 kD) monoclonal antibody clone OX-24 inhibits the binding of factor H to surface bound C3. This antibody recognizes the human serum complement protein factor H and a 43- 49kD truncated form of factor H present at low levels (1-5ug/ml) in plasma and urine. Factor H occurs on the surface of some myeloid cell lines. This antibody cross-reacts with sera from other primates (rhesus monkey, cynomolgus monkey, African green monkey) in immunoprecipitation assay, but not with cow, pig, sheep, chick or rabbit sera3. Reported applications of this antibody include flow cytometry, ELISA, immunoprecipitation and Western Blotting.

31965

CAA68704.1

P08603

139,096 Da

Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Complement Cascade Pathway (106405); Immune System Pathway (106386); Innate Immune System Pathway (106387); Regulation Of Complement Cascade Pathway (576254); Staphylococcus Aureus Infection Pathway (172846); Staphylococcus Aureus Infection Pathway (171867)

This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus repeat (SCR) domains. This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. Mutations in this gene have been associated with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic nephropathy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq, Oct 2011]

Function: Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway. Subcellular location: Secreted. Tissue specificity: Expressed by the liver and secreted in plasma. Involvement in disease: Basal laminar drusen (BLD) [MIM:126700]: Drusen are extracellular deposits that accumulate below the retinal pigment epithelium on Bruch membrane. Basal laminar drusen refers to an early adult-onset drusen phenotype that shows a pattern of uniform small, slightly raised yellow subretinal nodules randomly scattered in the macula. In later stages, these drusen often become more numerous, with clustered groups of drusen scattered throughout the retina. In time these small basal laminar drusen may expand and ultimately lead to a serous pigment epithelial detachment of the macula that may result in vision loss.Note: The gene represented in this entry is involved in disease pathogenesis.Complement factor H deficiency (CFHD) [MIM:609814]: A disorder that can manifest as several different phenotypes, including asymptomatic, recurrent bacterial infections, and renal failure. Laboratory features usually include decreased serum levels of factor H, complement component C3, and a decrease in other terminal complement components, indicating activation of the alternative complement pathway. It is associated with a number of renal diseases with variable clinical presentation and progression, including membranoproliferative glomerulonephritis and atypical hemolytic uremic syndrome.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.18 Ref.22 Ref.23 Ref.24 Ref.25 Ref.27 Ref.30 Ref.36Hemolytic uremic syndrome atypical 1 (AHUS1) [MIM:235400]: An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. Other genes may play a role in modifying the phenotype. Ref.19 Ref.20 Ref.21 Ref.26 Ref.28 Ref.29 Ref.30 Ref.38Age-related macular degeneration 4 (ARMD4) [MIM:610698]: A form of age-related macular degeneration, a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. Ref.40. Sequence similarities: Contains 20 Sushi (CCP/SCR) domains. Sequence caution: The sequence CAB41739.1 differs from that shown. Reason: Frameshift at position 341.

0.1 mg

245 USD