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Product Details
CF812134
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Mouse
/ IgG1
Class
Monoclonal
Type
Antibody
Clone
OTI7B3
Immunogen
Human recombinant protein fragment corresponding to amino acids 106-325 of human HADHA (NP_000173) produced in E.coli.
Conjugate
Form
Lyophilized
Concentration
See Label
Purification
Protein A/G
Storage buffer
PBS, pH 7.3, with 8% trehalose
Contains
no preservative
Storage conditions
-20° C, Avoid Freeze/Thaw Cycles
Shipping conditions
Wet ice
Target Information
This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit; 3-oxoacyl-CoA thiolase; 78 kDa gastrin-binding protein; gastrin-binding protein; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit; long-chain 2-enoyl-CoA hydratase; long-chain-3-hydroxyacyl-CoA dehydrogenase; mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit; mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit; mitochondrial trifunctional enzyme, alpha subunit; mitochondrial trifunctional protein, alpha subunit; Monolysocardiolipin acyltransferase; TP-alpha; Trifunctional enzyme subunit alpha, mitochondrial
Gene Aliases: C77020; ECHA; GBP; HADH; HADHA; LCEH; LCHAD; MTPA; RGD1560655; TP-ALPHA
UniProt ID: (Human) Q9UQC5, (Rat) Q64428, (Mouse) Q8BMS1
Entrez Gene ID: (Human) 3030, (Rat) 170670, (Mouse) 97212
fatty-acyl-CoA binding
3-hydroxyacyl-CoA dehydrogenase activity
acetyl-CoA C-acetyltransferase activity
enoyl-CoA hydratase activity
protein binding
long-chain-enoyl-CoA hydratase activity
long-chain-3-hydroxyacyl-CoA dehydrogenase activity
protein complex binding
NAD binding
acetyl-CoA C-acyltransferase activity
catalytic activity
oxidoreductase activity
lyase activity
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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