Alternative Name(s):

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

Species: (Organism)

Homo sapiens (Human)

Gene Names:

EHHADH

Tag info:

N-terminal 6xHis-SUMO-tagged

Target Protein AA Sequence:

MAEYTRLHNALALIRLRNPPVNAISTTLLRDIKEGLQKAVIDHTIKAIVICGAEGKFSAGADIRGFSAPRTFGLTLGHVVDEIQRNEKPVVAAIQGMAFGGGLELALGCHYRIAHAEAQVGLPEVTLGLLPGARGTQLLPRLTGVPAALDLITSGRRILADEALKLGILDKVVNSDPVEEAIRFAQRVSDQPLESRRLCNKPIQSLPNMDSIFSEALLKMRRQHPGCLAQEACVRAVQAAVQYPYEVGIKKEEELFLYLLQSGQARALQYAFFAERKANKWSTPSGASWKTASARPVSSVGVVGLGTMGRGIVISFARARIPVIAVDSDKNQLATANKMITSVLEKEASKMQQSGHPWSGPKPRLTSSVKELGGVDLVIEAVFEEMSLKKQVFAELSAVCKPEAFLCTNTSALDVDEIASSTDRPHLVIGTHFFSPAHVMKLLEVIPSQYSSPTTIATVMNLSKKIKKIGVVVGNCFGFVGNRMLNPYYNQAYFLLEEGSKPEEVDQVLEEFGFKMGPFRVSDLAGLDVGWKSRKGQGLTGPTLLPGTPARKRGNRRYCPIPDVLCELGRFGQKTGKGWYQYDKPLGRIHKPDPWLSKFLSRYRKTHHIEPRTISQDEILERCLYSLINEAFRILGEGIAASPEHIDVVYLHGYGWPRHKGGPMFYASTVGLPTVLEKLQKYYRQNPDIPQLEPSDYLKKLASQGNPPLKEWQSLAGSPSSKL

Expression Region:

1-723aa

Subcellular Location:

Peroxisome

Tissue Specificity:

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

Protein Length:

Full Length

Pathway:

PPARsignalingpathway

Mol. Weight:

95.5 kDa

Purity:

Greater than 90% as determined by SDS-PAGE.

Form:

Liquid or Lyophilized powder

Buffer:

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Research Areas:

Metabolism

Function:

Involvement in disease:

Fanconi renotubular syndrome 3 (FRTS3)

Relevance:

Reconstitution:

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Protein Families:

Enoyl-CoA hydratase/isomerase family; 3-hydroxyacyl-CoA dehydrogenase family

Reference:

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)