Recombinant Human Peroxisomal bifunctional enzyme (EHHADH) | CSB-EP600104HU

Recombinant Human Peroxisomal bifunctional enzyme (EHHADH) | CSB-EP600104HU | Cusabio

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

Gene Names: EHHADH

Research Areas: Metabolism

Organism: Homo sapiens (Human)

AA Sequence: MAEYTRLHNALALIRLRNPPVNAISTTLLRDIKEGLQKAVIDHTIKAIVICGAEGKFSAGADIRGFSAPRTFGLTLGHVVDEIQRNEKPVVAAIQGMAFGGGLELALGCHYRIAHAEAQVGLPEVTLGLLPGARGTQLLPRLTGVPAALDLITSGRRILADEALKLGILDKVVNSDPVEEAIRFAQRVSDQPLESRRLCNKPIQSLPNMDSIFSEALLKMRRQHPGCLAQEACVRAVQAAVQYPYEVGIKKEEELFLYLLQSGQARALQYAFFAERKANKWSTPSGASWKTASARPVSSVGVVGLGTMGRGIVISFARARIPVIAVDSDKNQLATANKMITSVLEKEASKMQQSGHPWSGPKPRLTSSVKELGGVDLVIEAVFEEMSLKKQVFAELSAVCKPEAFLCTNTSALDVDEIASSTDRPHLVIGTHFFSPAHVMKLLEVIPSQYSSPTTIATVMNLSKKIKKIGVVVGNCFGFVGNRMLNPYYNQAYFLLEEGSKPEEVDQVLEEFGFKMGPFRVSDLAGLDVGWKSRKGQGLTGPTLLPGTPARKRGNRRYCPIPDVLCELGRFGQKTGKGWYQYDKPLGRIHKPDPWLSKFLSRYRKTHHIEPRTISQDEILERCLYSLINEAFRILGEGIAASPEHIDVVYLHGYGWPRHKGGPMFYASTVGLPTVLEKLQKYYRQNPDIPQLEPSDYLKKLASQGNPPLKEWQSLAGSPSSKL

Source: E.coli

Tag Info: N-terminal 6xHis-SUMO-tagged

Expression Region: 1-723aa

Sequence Info: Full Length

MW: 95.5 kDa

Purity: Greater than 90% as determined by SDS-PAGE.

Relevance:

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)

Storage: The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself. Generally, the shelf life of liquid form is 6 months at -20?/-80?. The shelf life of lyophilized form is 12 months at -20?/-80?.

Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4? for up to one week.

Function:

Involvement in disease: Fanconi renotubular syndrome 3 (FRTS3)

Subcellular Location: Peroxisome

Protein Families: Enoyl-CoA hydratase/isomerase family; 3-hydroxyacyl-CoA dehydrogenase family

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

Paythway: PPARsignalingpathway

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.

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.

Uniprot ID: Q08426

HGNC Database Link: HGNC

UniGene Database Link: UniGene

KEGG Database Link: KEGG

STRING Database Link: STRING

OMIM Database Link: OMIM