Alternative Name(s):

Phytanic acid oxidase Phytanoyl-CoA alpha-hydroxylase

Species: (Organism)

Homo sapiens (Human)

Gene Names:

PHYH

Tag info:

N-terminal GST-tagged

Target Protein AA Sequence:

SGTISSASFHPQQFQYTLDNNVLTLEQRKFYEENGFLVIKNLVPDADIQRFRNEFEKICRKEVKPLGLTVMRDVTISKSEYAPSEKMITKVQDFQEDKELFRYCTLPEILKYVECFTGPNIMAMHTMLINKPPDSGKKTSRHPLHQDLHYFPFRPSDLIVCAWTAMEHISRNNGCLVVLPGTHKGSLKPHDYPKWEGGVNKMFHGIQDYEENKARVHLVMEKGDTVFFHPLLIHGSGQNKTQGFRKAISCHFASADCHYIDVKGTSQENIEKEVVGIAHKFFGAENSVNLKDIWMFRARLVKGERTNL

Expression Region:

1-338aa

Subcellular Location:

Peroxisome

Tissue Specificity:

Expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.

Protein Length:

Full Length

Pathway:

Mol. Weight:

62.4 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:

Signal Transduction

Function:

Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.

Involvement in disease:

Refsum disease (RD)

Relevance:

Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.

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:

PhyH family

Reference:

"Identification of PAHX, a Refsum disease gene." Mihalik S.J., Morrell J.C., Kim D., Sachsteder K.A., Watkins P.A., Gould S.J. Nat. Genet. 17:185-189(1997)