Key features and details
- Mouse monoclonal [5F3] to ERAB
- Suitable for: IHC-P
- Reacts with: Human
- Isotype: IgG1
- 倫理基準に準拠 - アニマル・フリーの生産
製品名Anti-ERAB antibody [5F3]
ERAB 一次抗体 製品一覧
製品の詳細Mouse monoclonal [5F3] to ERAB
特異性This antibody detects the recombinant human ERAB protein (27kDa) and recognizes the endogenous ERAB protein in cell extracts with virtually no crossreactivity with other human proteins.
アプリケーション適用あり: IHC-Pmore details
Recombinant, full-length Human ERAB protein (fusion protein).
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保存方法Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Concentration information loading...
特記事項（精製）Ammonium sulfate precipitated and dialyzed tissue culture supernatant.
The Abpromise guarantee
1/100 - 1/1000.
1/100 - 1/1000.
機能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).
組織特異性Expressed in normal tissues but is overexpressed in neurons affected in AD.
関連疾患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.
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.
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.
配列類似性Belongs to the short-chain dehydrogenases/reductases (SDR) family.
- Information by UniProt
- 17 beta hydroxysteroid dehydrogenase 10 antibody
- 17 beta hydroxysteroid dehydrogenase type 10 antibody
- 17-beta-HSD 10 antibody
IHC staining of purifiedab10260 on formalin-fixed paraffin-embedded human colon tissue. The tissue was incubated with 10 µg/ml of the primary antibody for 60 minutes at room temperature. A HRP kit was used for detection followed by hematoxylin counterstaining, according to the protocol provided. The image was captured with a 40X objective. Scale bar: 50 µm
ab10260 は 6 報の論文で使用されています。
- Liu L et al. Deacetylation of HSD17B10 by SIRT3 regulates cell growth and cell resistance under oxidative and starvation stresses. Cell Death Dis 11:563 (2020). PubMed: 32703935
- Xiao X et al. ABAD/17ß-HSD10 reduction contributes to the protective mechanism of huperzine a on the cerebral mitochondrial function in APP/PS1 mice. Neurobiol Aging 81:77-87 (2019). PubMed: 31252207
- Bertolin G et al. The TOMM machinery is a molecular switch in PINK1 and PARK2/PARKIN-dependent mitochondrial clearance. Autophagy 9:1801-17 (2013). ICC/IF ; Human . PubMed: 24149440
- Kitchens CA et al. Identification of chemosensitivity nodes for vinblastine through small interfering RNA high-throughput screens. J Pharmacol Exp Ther 339:851-8 (2011). Human . PubMed: 21880871
- Onoguchi K et al. Virus-infection or 5'ppp-RNA activates antiviral signal through redistribution of IPS-1 mediated by MFN1. PLoS Pathog 6:e1001012 (2010). WB, ICC/IF ; Human . PubMed: 20661427
- Stevanin G et al. Mutations in SPG11, encoding spatacsin, are a major cause of spastic paraplegia with thin corpus callosum. Nat Genet 39:366-372 (2007). PubMed: 17322883