BCKDHB FISH Probe
产品名称: BCKDHB FISH Probe
英文名称: BCKDHB FISH Probe
产品编号: FA0171
产品价格: 0
产品产地: 台湾
品牌商标: Abnova
更新时间: null
使用范围: null
亚诺法生技股份有限公司(Abnova)
- 联系人 :
- 地址 : 台湾台北市内湖区洲子街 108 号 9 楼
- 邮编 : 11493
- 所在区域 : 台湾
- 电话 : +886-920**1152 点击查看
- 传真 : 点击查看
- 邮箱 : sales@abnova.com.tw
- Specification
- Product Description:
- Made to order FISH probes for identification of gene amplification using Fluorescent In Situ Hybridization Technique. (Technology)
- Storage Instruction:
- Store at 4°C in the dark.
- Supplied Product:
- DAPI Counterstain (1500 ng/mL ) 250 uL
- Origin:
- Human
- Source:
- Genomic DNA
- Regulation Status:
- For research use only (RUO)
- Applications
- Fluorescent In Situ Hybridization (Cell)
- Protocol Download
- Application Image
- Fluorescent In Situ Hybridization (Cell)
- Entrez GeneID:
- 594
- Gene Name:
- BCKDHB
- Gene Alias:
- E1B,FLJ17880,dJ279A18.1
- Gene Description:
- branched chain keto acid dehydrogenase E1, beta polypeptide
- Gene Ontology:
- Hyperlink
- Gene Summary:
- Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq
- Other Designations:
- 2-oxoisovalerate dehydrogenase beta subunit,E1b-beta subunit of the branched-chain complex,OTTHUMP00000018048,OTTHUMP00000018049,branched chain alpha-ketoacid dehydrogenase E1-beta subunit,branched chain keto acid dehydrogenase E1 beta polypeptide,branche
- Related Disease