Recombinant Human Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial (ACADSB)

中文名稱：

Recombinant Human Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial(ACADSB)
貨號：

CSB-YP001128HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

Recombinant Human Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial(ACADSB)
貨號：

CSB-EP001128HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

Recombinant Human Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial(ACADSB)
貨號：

CSB-EP001128HU-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 Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial(ACADSB)
貨號：

CSB-BP001128HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

Recombinant Human Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial(ACADSB)
貨號：

CSB-MP001128HU
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

ACADSB
Uniprot No.：

P45954
別名：

2 MEBCAD; 2 methyl branched chain acyl CoA dehydrogenase; 2 methylbutyryl CoA dehydrogenase; 2 methylbutyryl coenzyme A dehydrogenase; 2-MEBCAD; 2-methyl branched chain acyl-CoA dehydrogenase; 2-methylbutyryl-CoA dehydrogenase; 2-methylbutyryl-coenzyme A dehydrogenase; ACAD7; ACADSB; ACDSB_HUMAN; acyl CoA dehydrogenase; short/branched chain; acyl Coenzyme A dehydrogenase short branched chain; mitochondrial; OTTHUMP00000020685; OTTHUMP00000046795; SBCAD; Short/branched chain specific acyl-CoA dehydrogenase; short/branched chain specific acyl-CoA dehydrogenase; mitochondrial
種屬：

Homo sapiens (Human)
蛋白長度：

Full Length of Mature Protein
表達區域：

34-432
氨基酸序列

KSSQSEA LLNITNNGIH FAPLQTFTDE EMMIKSSVKK FAQEQIAPLV STMDENSKME KSVIQGLFQQ GLMGIEVDPE YGGTGASFLS TVLVIEELAK VDASVAVFCE IQNTLINTLI RKHGTEEQKA TYLPQLTTEK VGSFCLSEAG AGSDSFALKT RADKEGDYYV LNGSKMWISS AEHAGLFLVM ANVDPTIGYK GITSFLVDRD TPGLHIGKPE NKLGLRASST CPLTFENVKV PEANILGQIG HGYKYAIGSL NEGRIGIAAQ MLGLAQGCFD YTIPYIKERI QFGKRLFDFQ GLQHQVAHVA TQLEAARLLT YNAARLLEAG KPFIKEASMA KYYASEIAGQ TTSKCIEWMG GVGYTKDYPV EKYFRDAKIG TIYEGASNIQ LNTIAKHIDA EY
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 Warning: in_array() expects parameter 2 to be array, null given in /www/web/cusabio_cn/public_html/caches/caches_template/default/content/show_product_protein.php on line 662
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：

Short and branched chain specific acyl-CoA dehydrogenase that catalyzes the removal of one hydrogen from C-2 and C-3 of the fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, acts specifically on short and branched chain acyl-CoA derivatives such as (S)-2-methylbutyryl-CoA as well as short straight chain acyl-CoAs such as butyryl-CoA. Plays an important role in the metabolism of L-isoleucine by catalyzing the dehydrogenation of 2-methylbutyryl-CoA, one of the steps of the L-isoleucine catabolic pathway. Can also act on valproyl-CoA, a metabolite of valproic acid, an antiepileptic drug.
基因功能參考文獻：
1. the c.1165 A>G mutation before knowing whether the optimal screening cut-off would minimize true positives or false negatives for SBCADD associated with this mutation. PMID: 23712021
2. Strong candidate gene for mitochondrial disease, based on recessive mutations detected in infantile patients PMID: 22277967
3. These findings confirm that SBCAD deficiency can be identified through newborn screening by acylcarnitine analysis. PMID: 20547083
4. Differences between the rat and human enzyme at positions 383, 222, and 220 alter substrate specificity without affecting substrate binding. PMID: 12855692
5. Identification and characterization of an IVS3+3A>G mutation (c.303+3A>G) in the SBCAD gene, and provide evidence that this mutation is disease-causing. PMID: 16317551
6. Results examine the mechanistic basis for dysfunction of the common variant short-chain acyl-CoA dehydrogenases and demonstrate that mutations can have a large impact on the redox properties of the enzyme. PMID: 16331963
7. Results indicate that substrate redox activation occurs in short-chain acyl-CoA dehydrogenase leading to a large enzyme midpoint potential shift. PMID: 16331964
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相關疾?。?/div>
Short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD)
亞細胞定位：

Mitochondrion matrix.
蛋白家族：

Acyl-CoA dehydrogenase family
組織特異性：

Ubiquitously expressed.
數據庫鏈接：

HGNC: 91

OMIM: 600301

KEGG: hsa:36

STRING: 9606.ENSP00000357873

UniGene: Hs.81934