HADHB Antibody
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中文名稱:HADHB兔多克隆抗體
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貨號:CSB-PA03035A0Rb
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規(guī)格:¥440
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促銷:
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圖片:
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其他:
產(chǎn)品詳情
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產(chǎn)品名稱:Rabbit anti-Homo sapiens (Human) HADHB Polyclonal antibody
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Uniprot No.:
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基因名:HADHB
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別名:2 enoyl Coenzyme A (CoA) hydratase beta subunit antibody; 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit antibody; 3 ketoacyl Coenzyme A thiolase antibody; 3 ketoacyl Coenzyme A thiolase of mitochondrial trifunctional protein beta subunit antibody; 3-ketoacyl-CoA thiolase antibody; Acetyl CoA acyltransferase antibody; Acetyl-CoA acyltransferase antibody; Beta ketothiolase antibody; Beta-ketothiolase antibody; ECHB antibody; ECHB_HUMAN antibody; HADH antibody; Hadhb antibody; Hydroxyacyl CoA dehydrogenase/3 ketoacyl CoA thiolase/enoyl CoA hydratase (trifunctional protein); beta subunit antibody; Hydroxyacyl Coenzyme A (CoA) dehydrogenase beta subunit antibody; Hydroxyacyl Coenzyme A dehydrogenase antibody; Hydroxyacyl Coenzyme A dehydrogenase beta subunit antibody; Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) beta subunit antibody; MGC87480 antibody; Mitochondrial trifunctional enzyme beta subunit antibody; Mitochondrial trifunctional protein beta subunit antibody; MSTP 029 antibody; MSTP029 antibody; MTPB antibody; TP beta antibody; TP-beta antibody; TPbeta antibody; Trifunctional enzyme subunit beta antibody; Trifunctional enzyme subunit beta mitochondrial antibody; Trifunctional protein antibody
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宿主:Rabbit
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反應(yīng)種屬:Human
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免疫原:Recombinant Human Trifunctional enzyme subunit beta, mitochondrial protein (35-283AA)
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免疫原種屬:Homo sapiens (Human)
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標(biāo)記方式:Non-conjugated
本頁面中的產(chǎn)品,HADHB Antibody (CSB-PA03035A0Rb),的標(biāo)記方式是Non-conjugated。對于HADHB Antibody,我們還提供其他標(biāo)記。見下表:
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克隆類型:Polyclonal
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抗體亞型:IgG
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純化方式:>95%, Protein G purified
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濃度:It differs from different batches. Please contact us to confirm it.
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保存緩沖液:Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 -
產(chǎn)品提供形式:Liquid
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應(yīng)用范圍:ELISA, IHC
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推薦稀釋比:
Application Recommended Dilution IHC 1:20-1:200 -
Protocols:
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儲(chǔ)存條件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期:Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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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.
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基因功能參考文獻(xiàn):
- Mutations in HADHB, which encodes the beta-subunit of mitochondrial trifunctional protein, cause infantile onset hypoparathyroidism and peripheral polyneuropathy. PMID: 24664533
- Heterozygous mutation in HADHB gene cause early-onset axonal axonal Charcot-Marie-tooth disease. PMID: 24314034
- 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
- The present findings showed that all missense mutations in HADHB were disease-causing. PMID: 19699128
- 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
- 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)疾病:Mitochondrial trifunctional protein deficiency (MTPD)
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亞細(xì)胞定位:Mitochondrion. Mitochondrion inner membrane. Mitochondrion outer membrane. Endoplasmic reticulum.
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蛋白家族:Thiolase family
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數(shù)據(jù)庫鏈接:
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