Name: HADHB Antibody
Brand: CUSABIO
SKU: CSB-PA03035A0Rb

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) HADHB Polyclonal antibody

Uniprot No.

P55084

Target Names

HADHB

Alternative Names

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

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human Trifunctional enzyme subunit beta, mitochondrial protein (35-283AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

The HADHB Antibody (Product code: CSB-PA03035A0Rb) is Non-conjugated. For HADHB Antibody with conjugates, please check the following table.

Conjugate	Product Code	Product Name	Application
HRP	CSB-PA03035B0Rb	HADHB Antibody, HRP conjugated	ELISA
FITC	CSB-PA03035C0Rb	HADHB Antibody, FITC conjugated
Biotin	CSB-PA03035D0Rb	HADHB Antibody, Biotin conjugated	ELISA

Clonality

Polyclonal

Isotype

IgG

Purification Method

>95%, Protein G purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4

Form

Liquid

Tested Applications

ELISA, IHC

Recommended Dilution

Application	Recommended Dilution
IHC	1:20-1:200

Protocols

ELISA Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

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

Lead Time

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.

Usage

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

Target Background

Function

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.

Gene References into Functions

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

Hide All

Involvement in disease

Mitochondrial trifunctional protein deficiency (MTPD)

Subcellular Location

Mitochondrion. Mitochondrion inner membrane. Mitochondrion outer membrane. Endoplasmic reticulum.

Protein Families

Thiolase family

Database Links

HGNC: 4803

OMIM: 143450

KEGG: hsa:3032

STRING: 9606.ENSP00000325136

UniGene: Hs.515848

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Code	CSB-PA03035A0Rb
Size	US$166
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HADHB Antibody

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