Name: ERAB Polyclonal Antibody
SKU: WP12230
Price: 268 USD
Availability: InStock

Product details

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],

Specifications

Size	50μL/100μL
Target	ERAB
Reactivity	Human, Mouse, Rat, Monkey
Applications	WB, IHC, IF, ELISA
MW(Calculated)
MW(Observed)	27kD
Host Species	Rabbit
Isotype	IgG
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	WB 1:500-1:2000; IHC 1:100-1:300; ELISA 1:40000; IF 1:50-200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Purification	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration	1 mg/ml
Clonality	Polyclonal
Clone Number
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.
Gene Name	HSD17B10
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
Species	Human
Gene ID	3028
Uniprot	Q99714,
Species.1	Mouse
Gene ID.1
Uniprot.1	O08756,
Species.2	Rat
Gene ID.2	63864
Uniprot.2	O70351
Cellular Localization	Mitochondrion . Mitochondrion matrix, mitochondrion nucleoid .
Tissue Expression	Ubiquitously expressed in normal tissues but is overexpressed in neurons affected in AD.
Signaling_pathway	Human Diseases >> Neurodegenerative disease >> Alzheimer disease;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases
Research Areas	>>Valine, leucine and isoleucine degradation; / >>Metabolic pathways; / >>Alzheimer disease; / >>Pathways of neurodegeneration - multiple diseases
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-CoA dehydrogenase deficiency (MHBD deficiency) [MIM:300438]. MHBD deficiency leads to neurological abnormalities, including psychomotor retardation, and, in virtually all patients, loss of mental and motor skills.,Disease:Defects in HSD17B10 are the cause of mental retardation syndromic X-linked type 10 (MRXS10) [MIM:300220]. MRXS10 is characterized by mild mental retardation, choreoathetosis and abnormal behavior.,Function:Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/RG9MTD1, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD).,similarity:Belongs to the short-chain dehydrogenases/reductases (SDR) family.,subunit:Homotetramer (By similarity). Interacts with MRPP1/RG9MTD1 and MRPP3/KIAA0391.,tissue specificity:Expressed in normal tissues but is overexpressed in neurons affected in AD.,

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ERAB Polyclonal Antibody

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ERAB Polyclonal Antibody

Product details

Specifications

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