HADHB Antibody

Code CSB-PA03035A0Rb
Size US$166
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  • Immunohistochemistry of paraffin-embedded human prostate tissue using CSB-PA03035A0Rb at dilution of 1:50
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) HADHB Polyclonal antibody
Uniprot No.
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.

Available Conjugates
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
Troubleshooting and 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.

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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
  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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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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