Rabbit anti-Human EHHADH Polyclonal Antibody | anti-EHHADH antibody

EHHADH (Peroxisomal Bifunctional Enzyme, PBE, PBFE, Enoyl-CoA Hydratase/3,2-trans-enoyl-CoA Isomerase, 3-hydroxyacyl-CoA Dehydrogenase, ECHD) (Biotin)

Cat. Num. AAA6377120

• Gene Names: EHHADH; LBP; ECHD; LBFP; PBFE; FRTS3; L-PBE
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: EHHADH; Polyclonal Antibody; EHHADH (Peroxisomal Bifunctional Enzyme; PBE; PBFE; Enoyl-CoA Hydratase/3; 2-trans-enoyl-CoA Isomerase; 3-hydroxyacyl-CoA Dehydrogenase; ECHD) (Biotin); anti-EHHADH antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human EHHADH.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-EHHADH antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Full length human EHHADH, aa1-723 (NP_001957.2).
• Immunogen Sequence: MAEYTRLHNALALIRLRNPPVNAISTTLLRDIKEGLQKAVIDHTIKAIVICGAEGKFSAGADIRGFSAPRTFGLTLGHVVDEIQRNEKPVVAAIQGMAFGGGLELALGCHYRIAHAEAQVGLPEVTLGLLPGARGTQLLPRLTGVPAALDLITSGRRILADEALKLGILDKVVNSDPVEEAIRFAQRVSDQPLESRRLCNKPIQSLPNMDSIFSEALLKMRRQHPGCLAQEACVRAVQAAVQYPYEVGIKKEEELFLYLLQSGQARALQYAFFAERKANKWSTPSGASWKTASARPVSSVGVVGLGTMGRGIVISFARARIPVIAVDSDKNQLATANKMITSVLEKEASKMQQSGHPWSGPKPRLTSSVKELGGVDLVIEAVFEEMSLKKQVFAELSAVCKPEAFLCTNTSALDVDEIASSTDRPHLVIGTHFFSPAHVMKLLEVIPSQYSSPTTIATVMNLSKKIKKIGVVVGNCFGFVGNRMLNPYYNQAYFLLEEGSKPEEVDQVLEEFGFKMGPFRVSDLAGLDVGWKSRKGQGLTGPTLLPGTPARKRGNRRYCPIPDVLCELGRFGQKTGKGWYQYDKPLGRIHKPDPWLSKFLSRYRKTHHIEPRTISQDEILERCLYSLINEAFRILGEGIAASPEHIDVVYLHGYGWPRHKGGPMFYASTVGLPTVLEKLQKYYRQNPDIPQLEPSDYLKKLASQGNPPLKEWQSLAGSPSSKL
• Conjugate: Biotin
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis of EHHADH expression in transfected 293T cell line by EHHADH polyclonal antibody. Lane 1: EHHADH transfected lysate (79.5kD). Lane 2: Non-transfected lysate.)

Related Product Information for anti-EHHADH antibody

EHHADH 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.

Product Categories/Family for anti-EHHADH antibody

Antibodies; Enzymes; Dehydrogenase

NCBI and Uniprot Product Information

• NCBI GI #: 68989263
• NCBI GeneID: 1962
• NCBI Accession #: NP_001957.2
• NCBI GenBank Nucleotide #: NM_001966.2
• UniProt Accession #: Q08426; Q58EZ5; A8K6Y3; B4DWG3; D3DNU0
• Molecular Weight: 69,154 Da
• NCBI Official Full Name: peroxisomal bifunctional enzyme isoform 1
• NCBI Official Synonym Full Names: enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase
• NCBI Official Symbol: EHHADH
• NCBI Official Synonym Symbols: LBP; ECHD; LBFP; PBFE; FRTS3; L-PBE
• NCBI Protein Information: peroxisomal bifunctional enzyme
• UniProt Protein Name: Peroxisomal bifunctional enzyme
• Protein Family: Peroxisomal bifunctional enzyme
• UniProt Gene Name: EHHADH
• UniProt Synonym Gene Names: ECHD; PBE; PBFE
• UniProt Entry Name: ECHP_HUMAN

NCBI Description

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. [provided by RefSeq, Oct 2009]

Uniprot Description

EHHADH: 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; Amino Acid Metabolism - tryptophan; Other Amino Acids Metabolism - beta-alanine; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 4.2.1.17; Secondary Metabolites Metabolism - limonene and pinene degradation; Carbohydrate Metabolism - butanoate; Oxidoreductase; Isomerase; Carbohydrate Metabolism - propanoate; Lipid Metabolism - fatty acid; Lyase; Amino Acid Metabolism - lysine degradation; EC 5.3.3.8; EC 1.1.1.35

Chromosomal Location of Human Ortholog: 3q26.3-q28

Cellular Component: mitochondrion; peroxisome; cytosol

Molecular Function: enzyme binding; dodecenoyl-CoA delta-isomerase activity; enoyl-CoA hydratase activity; coenzyme binding; 3-hydroxyacyl-CoA dehydrogenase activity; receptor binding

Biological Process: fatty acid beta-oxidation; internal protein amino acid acetylation

Disease: Fanconi Renotubular Syndrome 3