Molecular weight (DA)
27kD
Immunogen
The antiserum was produced against synthesized peptide derived from human ERAB. AA range:111-160
Specificity
ERAB Polyclonal Antibody detects endogenous levels of ERAB protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes 3-hydroxyacyl-CoA dehydrogenase type II, a member of the short-chain dehydrogenase/reductase superfamily. The gene product is a mitochondrial protein that catalyzes the oxidation of a wide variety of fatty acids and steroids, and is a subunit of mitochondrial ribonuclease P, which is involved in tRNA maturation. The protein has been implicated in the development of Alzheimer disease, and mutations in the gene are the cause of 17beta-hydroxysteroid dehydrogenase type 10 (HSD10) deficiency. Several alternatively spliced transcript variants have been identified, but the full-length nature of only two transcript variants has been determined. [provided by RefSeq, Aug 2014],
Function
catalytic activity:(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA + NAD(+) = 2-methylacetoacetyl-CoA + NADH.,catalytic activity:(S)-3-hydroxyacyl-CoA + NAD(+) = 3-oxoacyl-CoA + NADH.,disease:A chromosomal microduplication involving HSD17B10 and HUWE1 is the cause of mental retardation X-linked type 17 (MRX17) [MIM:300705]; also known as mental retardation X-linked type 31 (MRX31). Mental retardation is characterized by significantly sub-average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period. In contrast to syndromic or specific X-linked mental retardation which also present with associated physical, neurological and/or psychiatric manifestations, intellectual deficiency is the only primary symptom of non-syndromic X-linked mental retardation.,disease:Defects in HSD17B10 are the cause of 2-methyl-3-hydroxybutyryl-
Protein name
3-hydroxyacyl-CoA dehydrogenase type-2
Other name
HSD17B10; ERAB; HADH2; MRPP2; SCHAD; XH98G2; 3-hydroxyacyl-CoA dehydrogenase type-2; 17-beta-hydroxysteroid dehydrogenase 10; 17-beta-HSD 10; 3-hydroxy-2-methylbutyryl-CoA dehydrogenase; 3-hydroxyacyl-CoA dehydrogenase type II; Endoplasmic
Fields
>>Valine, leucine and isoleucine degradation;>>Metabolic pathways;>>Alzheimer disease;>>Pathways of neurodegeneration - multiple diseases
Human protein sequence Database
Q99714
Mouse protein sequence database
O08756
Rat protein sequence database
O70351
Cellular localization
Mitochondrion . Mitochondrion matrix, mitochondrion nucleoid .
Tissue expression
Ubiquitously expressed in normal tissues but is overexpressed in neurons affected in AD.
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