Recombinant Human 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (BCKDHB)

中文名稱：

Recombinant Human 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial(BCKDHB)
貨號：

CSB-YP002606HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

Recombinant Human 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial(BCKDHB)
貨號：

CSB-EP002606HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

Recombinant Human 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial(BCKDHB)
貨號：

CSB-EP002606HU-B
說明書：

MSDS Datasheet
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

Recombinant Human 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial(BCKDHB)
貨號：

CSB-BP002606HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

Recombinant Human 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial(BCKDHB)
貨號：

CSB-MP002606HU
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

BCKDHB
Uniprot No.：

P21953
別名：

2 oxoisovalerate dehydrogenase subunit beta mitochondrial; 2-oxoisovalerate dehydrogenase subunit beta; mitochondrial; BCKDE1B; BCKDH E1-beta; BCKDHB; Branched chain alpha ketoacid dehydrogenase E1 beta subunit; Branched chain keto acid dehydrogenase E1 beta polypeptide ; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; dJ279A18.1; E1B; E1b beta subunit of the branched chain complex; FLJ17880; ODBB_HUMAN; RP1-279A18.1
種屬：

Homo sapiens (Human)
蛋白長度：

Full length protein
表達區域：

51-392
氨基酸序列

VAHFTFQPDP EPREYGQTQK MNLFQSVTSA LDNSLAKDPT AVIFGEDVAF GGVFRCTVGL RDKYGKDRVF NTPLCEQGIV GFGIGIAVTG ATAIAEIQFA DYIFPAFDQI VNEAAKYRYR SGDLFNCGSL TIRSPWGCVG HGALYHSQSP EAFFAHCPGI KVVIPRSPFQ AKGLLLSCIE DKNPCIFFEP KILYRAAAEE VPIEPYNIPL SQAEVIQEGS DVTLVAWGTQ VHVIREVASM AKEKLGVSCE VIDLRTIIPW DVDTICKSVI KTGRLLISHE APLTGGFASE ISSTVQEECF LNLEAPISRV CGYDTPFPHI FEPFYIPDKW KCYDALRKMI NY
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產品評價

靶點詳情

功能：

The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
基因功能參考文獻：
1. Five novel mutations in BCKDHB were identified in MSUD patients. PMID: 26453840
2. we describe the presenting symptoms and clinical course of a Chinese boy with intermittent Intermittent maple syrup urine disease from BCKDHB gene mutation PMID: 26239723
3. Mutations in BCKDHB gene is associated with maple syrup urine disease. PMID: 23313820
4. analysis of roles of His291-alpha and His146-beta' in the reductive acylation reaction catalyzed by human branched-chain alpha-ketoacid dehydrogenase: refined phosphorylation loop structure in the active site PMID: 12902323
5. cross-talk between thiamin diphosphate binding and phosphorylation loop conformation has an effect on human branched-chain alpha-keto acid decarboxylase/dehydrogenase PMID: 15166214
6. A key tyrosine residue in the E1b active site functions as a conformational switch to reduce the reactivity of the thiamine diphosphate cofactor through interactions with its thiazolium ring. PMID: 16472748
7. the two active sites in the E1b heterotetramer operate independently during the ThDP-dependent decarboxylation reaction PMID: 17329260
8. Reconstructed haplotype from BCKDHB variants was associated with premature ovarian failure. PMID: 17524396
9. Founder mutation in Ashkenazi Jewish population that causes maple syrup urine disease. PMID: 11509994
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相關疾病：

Maple syrup urine disease 1B (MSUD1B)
亞細胞定位：

Mitochondrion matrix.
數據庫鏈接：

HGNC: 987

OMIM: 248600

KEGG: hsa:594

STRING: 9606.ENSP00000318351

UniGene: Hs.156414