Rabbit ERAB Polyclonal Antibody | anti-ERAB antibody

ERAB (E135) Polyclonal Antibody

Cat. Num. AAA3001565

• Gene Names: HSD17B10; ABAD; CAMR; ERAB; HCD2; MHBD; HADH2; MRPP2; MRX17; MRX31; SCHAD; MRXS10; SDR5C1; HSD10MD; 17b-HSD10; DUPXp11.22
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: ERAB; Polyclonal Antibody; ERAB (E135) Polyclonal Antibody; 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 reticulum-associated amyloid beta-peptide-binding protein; Mitochondrial ribonuclease P protein 2; Mitochondrial RNase P protein 2; Short-chain type dehydrogenase/reductase XH98G2; Type II HADH; HSD17B10; HADH2; MRPP2; SCHAD; XH98G2; anti-ERAB antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: ERAB (E135) polyclonal antibody detects endogenous levels of ERAB protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 252

• Applicable Applications for anti-ERAB antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500-1:1000; IHC: 1:50-1:200
• Immunogen: Synthetic peptide, corresponding to amino acids 102-150 of Human ERAB.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of ERAB (E135) polyclonal antibody at 1:500 dilutionLane1:HEK293T whole cell lysateLane2:Raw264. 7 whole cell lysateLane3:PC12 whole cell lysate)

Immunohistochemistry (IHC)

(Immunohistochemistry (IHC) analyzes of ERAB (E135) pAb in paraffin-embedded human breast carcinoma tissue at 1:50.)

Western Blot (WB)

(Western Blot (WB) analysis of ERAB (E135) pAb at 1:500 dilutionLane1:HEK293T whole cell lysate (40ug) Lane2:K562 whole cell lysate (40ug) Lane3:C6 whole cell lysate (40ug) Lane4:AML-12 whole cell lysate (40ug) Lane5:H9C2 whole cell lysate (40ug))

Related Product Information for anti-ERAB antibody

beta-Amyloid is a neurotoxic peptide that is associated with the pathogenesis of Alzheimer's disease. beta-Amyloid aggregates induce cell death of neurons through the disruption of cell membranes and the generation of reactive oxygen intermediates.These neurotoxic effects are also attributed to the interaction of beta-Amyloid with intracellular proteins, specifically ERAB, the endoplasmic reticulum-associated beta-Amyloid-binding protein. ERAB is characterized as a NAD+-dependent dehydrogenase that is constitutively expressed in tissues and overexpressed in neurons affected in Alzheimer's disease. Cells overexpressing ERAB in vitro have been shown to be more sensitive to beta-Amyloid-induced stress, and blocking the activity of ERAB has been shown to inhibit this cell death, indicating that beta-Amyloid induced cell death is mediated by ERAB.

NCBI and Uniprot Product Information

• NCBI GI #: 83715985
• NCBI GeneID: 3028
• NCBI Accession #: NP_001032900.1
• NCBI GenBank Nucleotide #: NP_001032900.1
• UniProt Accession #: Q99714
• Molecular Weight: ~ 27kDa
• NCBI Official Full Name: 3-hydroxyacyl-CoA dehydrogenase type-2 isoform 2
• NCBI Official Synonym Full Names: hydroxysteroid 17-beta dehydrogenase 10
• NCBI Official Symbol: HSD17B10
• NCBI Official Synonym Symbols: ABAD; CAMR; ERAB; HCD2; MHBD; HADH2; MRPP2; MRX17; MRX31; SCHAD; MRXS10; SDR5C1; HSD10MD; 17b-HSD10; DUPXp11.22
• NCBI Protein Information: 3-hydroxyacyl-CoA dehydrogenase type-2
• UniProt Protein Name: 3-hydroxyacyl-CoA dehydrogenase type-2
• Protein Family: Erabutoxin
• UniProt Gene Name: HSD17B10
• UniProt Synonym Gene Names: ERAB; HADH2; MRPP2; SCHAD; XH98G2; 17-beta-HSD 10; Mitochondrial RNase P protein 2
• UniProt Entry Name: HCD2_HUMAN

NCBI Description

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]

Uniprot Description

HADH2: Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, 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). Defects in HSD17B10 are the cause of 2-methyl-3- hydroxybutyryl-CoA dehydrogenase deficiency (MHBD deficiency). MHBD deficiency leads to neurological abnormalities, including psychomotor retardation, and, in virtually all patients, loss of mental and motor skills. Defects in HSD17B10 are the cause of mental retardation syndromic X-linked type 10 (MRXS10). MRXS10 is characterized by mild mental retardation, choreoathetosis and abnormal behavior. A chromosomal microduplication involving HSD17B10 and HUWE1 is the cause of mental retardation X-linked type 17 (MRX17); 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. Belongs to the short-chain dehydrogenases/reductases (SDR) family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 1.1.1.178; Oxidoreductase; Mitochondrial; EC 1.1.1.51; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.1.1.35

Chromosomal Location of Human Ortholog: Xp11.2

Cellular Component: mitochondrion; endoplasmic reticulum; mitochondrial matrix; cytoplasm; mitochondrial inner membrane; plasma membrane

Molecular Function: 3(or 17)beta-hydroxysteroid dehydrogenase activity; protein binding; 7-alpha-hydroxysteroid dehydrogenase activity; 3-hydroxy-2-methylbutyryl-CoA dehydrogenase activity; 3-hydroxyacyl-CoA dehydrogenase activity

Biological Process: tRNA processing; branched chain family amino acid catabolic process; lipid metabolic process

Disease: Mental Retardation, X-linked, Syndromic 10; 17-beta-hydroxysteroid Dehydrogenase X Deficiency