Trifunctional enzyme subunit beta, mitochondrial Polyclonal Antibody

Catalog Number:

42383

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#: P55084

Form of Antibody:

Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4

Storage/Stability:

Immunogen:

Recombinant human Trifunctional enzyme subunit beta, mitochondrial protein

Purification:

Caprylic Acid Ammonium Sulfate Precipitation purified

Specificity/Sensitivity:

The antibody detects endogenous level of total Trifunctional enzyme subunit beta, mitochondrial polyclonal antibody.

Applications:

IHC

Background:

Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex.

References:

[1] "Structural analysis of cDNAs for subunits of human mitochondrial fatty acid beta-oxidation trifunctional protein."Kamijo T., Aoyama T., Komiyama A., Hashimoto T.Biochem. Biophys. Res. Commun. 199:818-825(1994) [2] "Genomic and mutational analysis

appl_detail:

Immunohistochemistry: 1:20 - 1:200

Datasheet PDF