Peroxisomal bifunctional enzyme Recombinant Protein | EHHADH recombinant protein

Recombinant Human Peroxisomal bifunctional enzyme

Cat. Num. AAA1455224

• Gene Names: EHHADH; LBP; ECHD; LBFP; PBFE; FRTS3; L-PBE
• Purity: Greater or equal to 85% purity as determined by SDS-PAGE.
• Synonyms: Peroxisomal bifunctional enzyme; Recombinant Human Peroxisomal bifunctional enzyme; EHHADH recombinant protein

• Host: E Coli or Yeast or Baculovirus or Mammalian Cell
• Purity/Purification: Greater or equal to 85% purity as determined by SDS-PAGE.
• Form/Format: Lyophilized or liquid (Format to be determined during the manufacturing process)
• Sequence Positions: 1-723aa; Full Length
• Sequence: MAEYTRLHNALALIRLRNPPVNAISTTLLRDIKEGLQKAVIDHTIKAIVICGAEGKFSAGADIRGFSAPRTFGLTLGHVVDEIQRNEKPVVAAIQGMAFGGGLELALGCHYRIAHAEAQVGLPEVTLGLLPGARGTQLLPRLTGVPAALDLITSGRRILADEALKLGILDKVVNSDPVEEAIRFAQRVSDQPLESRRLCNKPIQSLPNMDSIFSEALLKMRRQHPGCLAQEACVRAVQAAVQYPYEVGIKKEEELFLYLLQSGQARALQYAFFAERKANKWSTPSGASWKTASARPVSSVGVVGLGTMGRGIVISFARARIPVIAVDSDKNQLATANKMITSVLEKEASKMQQSGHPWSGPKPRLTSSVKELGGVDLVIEAVFEEMSLKKQVFAELSAVCKPEAFLCTNTSALDVDEIASSTDRPHLVIGTHFFSPAHVMKLLEVIPSQYSSPTTIATVMNLSKKIKKIGVVVGNCFGFVGNRMLNPYYNQAYFLLEEGSKPEEVDQVLEEFGFKMGPFRVSDLAGLDVGWKSRKGQGLTGPTLLPGTPARKRGNRRYCPIPDVLCELGRFGQKTGKGWYQYDKPLGRIHKPDPWLSKFLSRYRKTHHIEPRTISQDEILERCLYSLINEAFRILGEGIAASPEHIDVVYLHGYGWPRHKGGPMFYASTVGLPTVLEKLQKYYRQNPDIPQLEPSDYLKKLASQGNPPLKEWQSLAGSPSSKL
• Sequence Length: 627

• Preparation and Storage: Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

SDS-PAGE

Product Categories/Family for EHHADH recombinant protein

Metabolism

References

Import of human bifunctional enzyme into peroxisomes of human hepatoma cells in vitro.Chen G.L., Balfe A., Erwa W., Hoefler G., Gaertner J., Aikawa J., Chen W.W.Biochem. Biophys. Res. Commun. 178:1084-1091(1991)
Peroxisomal beta-oxidation enzyme proteins in adrenoleukodystrophy distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy.Chen W.W., Watkins P.A., Osumi T., Hashimoto T., Moser H.W.Proc. Natl. Acad. Sci. U.S.A. 84:1425-1428(1987)
Lysine acetylation targets protein complexes and co-regulates major cellular functions.Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.Science 325:834-840(2009)
Regulation of cellular metabolism by protein lysine acetylation.Zhao S., Xu W., Jiang W., Yu W., Lin Y., Zhang T., Yao J., Zhou L., Zeng Y., Li H., Li Y., Shi J., An W., Hancock S.M., He F., Qin L., Chin J., Yang P., Chen X., Lei Q., Xiong Y., Guan K.L.Science 327:1000-1004(2010)
Initial characterization of the human central proteome.Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.BMC Syst. Biol. 5:17-17(2011)
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.J. Proteomics 96:253-262(2014)
Mistargeting of peroxisomal EHHADH and inherited renal Fanconi's syndrome.Klootwijk E.D., Reichold M., Helip-Wooley A., Tolaymat A., Broeker C., Robinette S.L., Reinders J., Peindl D., Renner K., Eberhart K., Assmann N., Oefner P.J., Dettmer K., Sterner C., Schroeder J., Zorger N., Witzgall R., Reinhold S.W., Stanescu H.C., Bockenhauer D., Jaureguiberry G., Courtneidge H., Hall A.M., Wijeyesekera A.D., Holmes E., Nicholson J.K., O'Brien K., Bernardini I., Krasnewich D.M., Arcos-Burgos M., Izumi Y., Nonoguchi H., Jia Y., Reddy J.K., Ilyas M., Unwin R.J., Gahl W.A., Warth R., Kleta R.N. Engl. J. Med. 370:129-138(2014)

NCBI and Uniprot Product Information

• NCBI GI #: 261878539
• NCBI GeneID: 1962
• NCBI Accession #: NP_001159887.1
• NCBI GenBank Nucleotide #: NM_001166415.1
• UniProt Accession #: Q08426; Q58EZ5; A8K6Y3; B4DWG3; D3DNU0
• Molecular Weight: 95.5 kDa
• NCBI Official Full Name: peroxisomal bifunctional enzyme isoform 2
• NCBI Official Synonym Full Names: enoyl-CoA, hydratase/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: EC 5.3.3.8; EC 4.2.1.17; Carbohydrate Metabolism - propanoate; Isomerase; Amino Acid Metabolism - lysine degradation; Mitochondrial; Other Amino Acids Metabolism - beta-alanine; Oxidoreductase; Lipid Metabolism - fatty acid; Secondary Metabolites Metabolism - limonene and pinene degradation; Lyase; Carbohydrate Metabolism - butanoate; EC 1.1.1.35; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - valine, leucine and isoleucine degradation

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

Cellular Component: cytosol; mitochondrion; peroxisome

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

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

Disease: Fanconi Renotubular Syndrome 3