Recombinant Human PCBD1 Protein (His Tag)

Specifications

Catalog number

PKSH032981-01

Size / count

10 µg

Dry ice

No

Hazardous

No

Discontinued

No

Background

Pterin-4-α-Carbinolamine Dehydratase (PCBD1) is the founding member of the Pterin-4-α-Carbinolamine Dehydratase Family. PCBD1 is involved in Tetrahydrobiopterin biosynthesis. It seems to prevent the formation of 7-Pterins and accelerate the formation of Quinonoid-BH2. Furthermore, PCBD1 regulates the homodimerization of the transcription factor Hepatocyte Nuclear Factor 1 (HNF1) and enhances its transcriptional activity. Defects in PCBD1 are the cause of BH4-Deficient Hyperphenylalaninemia Type D (HPABH4D) . HPABH4D is characterized by the excretion of 7-substituted Pterins in the urine of affected patients.

Abbreviation

PCBD1

Synonyms

4-Alpha-Hydroxy-Tetrahydropterin Dehydratase; DCoH; Dimerization Cofactor of HNF1; Dimerization Cofactor of Hepatocyte Nuclear Factor 1-Alpha; PHS; Phenylalanine Hydroxylase-Stimulating Protein; Pterin Carbinolamine Dehy; Pterin-4-Alpha-Carbinolamine Dehydratase

UniProt

P61457

Accession Number

P61457

Expression System

E.coli

Tag

N-His

Sequence

Ala2-Thr104

Field of Research

Signal Transduction; epigenetics and nuclear signal; metabolism

Endotoxin

< 1.0 EU per μg of the protein as determined by the LAL method.

Purity

> 95 % as determined by reducing SDS-PAGE.

Bioactivity

Not validated for activity

Reconstitution

Not Applicable

Shipping Conditions

This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs. Upon receipt, store it immediately at < - 20°C.

Storage Conditions

Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles.

Calculated Molecular Weight

14.2 kDa

Observed Molecular Weight

14 kDa

Formulation

Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, 1mM DTT, pH 8.0.

Species

Human