Recombinant Human Trifunctional enzyme subunit beta, mitochondrial (HADHB), partial

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中文名稱：

人HADHB重組蛋白
品名簡稱：

Recombinant Human HADHB protein, partial
貨號：

CSB-EP347003HU1
說明書：

MSDS Datasheet
規(guī)格：

￥1344
圖片：
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

純度：

Greater than 90% as determined by SDS-PAGE.
基因名：

HADHB
Uniprot No.：

P55084
別名：

2 enoyl Coenzyme A (CoA) hydratase beta subunit; 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit; 3 ketoacyl Coenzyme A thiolase; 3 ketoacyl Coenzyme A thiolase of mitochondrial trifunctional protein beta subunit; 3-ketoacyl-CoA thiolase; Acetyl CoA acyltransferase; Acetyl-CoA acyltransferase; Beta ketothiolase; Beta-ketothiolase; ECHB; ECHB_HUMAN; HADH; Hadhb; Hydroxyacyl CoA dehydrogenase/3 ketoacyl CoA thiolase/enoyl CoA hydratase (trifunctional protein); beta subunit; Hydroxyacyl Coenzyme A (CoA) dehydrogenase beta subunit; Hydroxyacyl Coenzyme A dehydrogenase; Hydroxyacyl Coenzyme A dehydrogenase beta subunit; Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) beta subunit; MGC87480; Mitochondrial trifunctional enzyme beta subunit; Mitochondrial trifunctional protein beta subunit; MSTP 029; MSTP029; MTPB; TP beta; TP-beta; TPbeta; Trifunctional enzyme subunit beta; Trifunctional enzyme subunit beta mitochondrial; Trifunctional protein
種屬：

Homo sapiens (Human)
蛋白長度：

Partial
來源：

E.coli
分子量：

53.8kDa
表達(dá)區(qū)域：

35-283aa
氨基酸序列

APAVQTKTKKTLAKPNIRNVVVVDGVRTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMRKLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLEQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRP
Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
蛋白標(biāo)簽：

N-terminal GST-tagged
產(chǎn)品提供形式：

Liquid or 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.
緩沖液：

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
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(zhì)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：

3-7 business days
注意事項：

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

Please contact us to get it.

產(chǎn)品評價

靶點(diǎn)詳情

功能：

Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway. The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA. Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids. Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities, while the trifunctional enzyme subunit beta/HADHB described here bears the 3-ketoacyl-CoA thiolase activity.
基因功能參考文獻(xiàn)：
1. Mutations in HADHB, which encodes the beta-subunit of mitochondrial trifunctional protein, cause infantile onset hypoparathyroidism and peripheral polyneuropathy. PMID: 24664533
2. Heterozygous mutation in HADHB gene cause early-onset axonal axonal Charcot-Marie-tooth disease. PMID: 24314034
3. mutational analysis of the HADHB gene, which encodes long-chain 3-ketoacyl-CoA thiolase, identified compound heterozygous mutations of c.520C>T (p.R141C) and c.1331G>A (p.R411K) in a case of mitochondrial trifunctional protein deficiency PMID: 22000755
4. The present findings showed that all missense mutations in HADHB were disease-causing. PMID: 19699128
5. HADHB trifunctional enzyme, human renin, and poly(C)-binding protein are novel renin mRNA-binding proteins that target a cis-element in the 3'-UTR of renin mRNA and regulate renin production PMID: 12933794
6. The common disease causing mutation of G1528C in MTP gene in caucasian in probably not a common mutation in Chinese Han people in Beijing. PMID: 17199921
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相關(guān)疾?。?/div>
Mitochondrial trifunctional protein deficiency (MTPD)
亞細(xì)胞定位：

Mitochondrion. Mitochondrion inner membrane. Mitochondrion outer membrane. Endoplasmic reticulum.
蛋白家族：

Thiolase family
數(shù)據(jù)庫鏈接：

HGNC: 4803

OMIM: 143450

KEGG: hsa:3032

STRING: 9606.ENSP00000325136

UniGene: Hs.515848