Recombinant Human Peroxisomal bifunctional enzyme (EHHADH)

Cat.No	ACP03990	Target Name	EHHADH
Target Synonyms	3 hydroxyacyl CoA dehydrogenase; 3	Form	Liquid or Lyophilized powder
Expression System	E.coli	Expression Range	1-723aa
Mol Weight	95.5kDa	Protein Length	Full length
Purity	Greater than 90% as determined by SDS-PAGE.	Storage Buffer	5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Cat.No

ACP03990

Target Name

EHHADH

Target Synonyms

3 hydroxyacyl CoA dehydrogenase; 3

Form

Liquid or Lyophilized powder

Expression System

E.coli

Expression Range

1-723aa

Mol Weight

95.5kDa

Protein Length

Full length

Purity

Greater than 90% as determined by SDS-PAGE.

Storage Buffer

5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Target Species	Human	Uniprot ID	Q08426

Background Information

Uniprot Id

Q08426
Target Species

Human
Target Name

EHHADH
Target Full Name

Peroxisomal bifunctional enzyme
Target Function

Peroxisomal trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities. Catalyzes two of the four reactions of the long straight chain fatty acids peroxisomal beta-oxidation pathway. Optimal isomerase for 2,5 double bonds into 3,5 form isomerization in a range of enoyl-CoA species (Probable). Also able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species. With HSD17B4, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity. Regulates the amount of medium-chain dicarboxylic fatty acids which are essential regulators of all fatty acid oxidation pathways. Also involved in the degradation of long-chain dicarboxylic acids through peroxisomal beta-oxidation.
Target Involvement

Fanconi renotubular syndrome 3 (FRTS3)
Target Subcellular Location

Peroxisome.
Target Protein Families

Enoyl-CoA hydratase/isomerase family; 3-hydroxyacyl-CoA dehydrogenase family
Target Tissue Specificity

Liver and kidney. Strongly expressed in the terminal segments of the proximal tubule. Lower amounts seen in the brain.
Target Research Area

Metabolism
Target Synonyms

3 hydroxyacyl CoA dehydrogenase; 3,2 trans enoyl CoA isomerase; 3-hydroxyacyl-CoA dehydrogenase; ECHD; ECHP_HUMAN; EHHADH; Enoyl Coenzyme A; Enoyl Coenzyme A; hydratase/3 hydroxyacyl Coenzyme A dehydrogenase ; L 3 hydroxyacyl CoA dehydrogenase; L bifunctional protein; L bifunctional protein; peroxisomal; L PBE; LBFP; LBP; MGC120586; MS730; PBE; PBFE; Peroxisomal bifunctional enzyme; Peroxisomal enoyl CoA hydratase
Target Background

The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defects in this gene are a cause of peroxisomal disorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for this gene.

Recombinant Human Peroxisomal bifunctional enzyme (EHHADH)

Specifications

Immunogen Information

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