HADHB Antibody

中文名稱：

HADHB兔多克隆抗體
貨號：

CSB-PA03035A0Rb
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human prostate tissue using CSB-PA03035A0Rb at dilution of 1:50
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) HADHB Polyclonal antibody
Uniprot No.：

P55084
基因名：

HADHB
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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Trifunctional enzyme subunit beta, mitochondrial protein (35-283AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，HADHB Antibody (CSB-PA03035A0Rb)，的標記方式是Non-conjugated。對于HADHB Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA03035B0Rb	HADHB Antibody, HRP conjugated	ELISA
FITC	CSB-PA03035C0Rb	HADHB Antibody, FITC conjugated
Biotin	CSB-PA03035D0Rb	HADHB Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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：
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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相關疾病：

Mitochondrial trifunctional protein deficiency (MTPD)
亞細胞定位：

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

Thiolase family
數據庫鏈接：

HGNC: 4803

OMIM: 143450

KEGG: hsa:3032

STRING: 9606.ENSP00000325136

UniGene: Hs.515848

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