Recombinant Human Fumarylacetoacetase/FAH Protein (His Tag)

Specifications

Catalog number

PKSH032463-01

Size / count

10 µg

Dry ice

No

Hazardous

No

Discontinued

No

Background

Fumarylacetoacetase belongs to the FAH family. Fumarylacetoacetase is primary expressed in liver and kidney. It exists as a homodimer and catalyzes the hydrolysis of 4-fumarylacetoacetate into fumarate and acetoacetate. Defects in Fumarylacetoacetase cause tyrosinemia type 1, which is congenital metabolism defect characterized by elevated levels of tyrosine in the blood and urine, and hepatorenal manifestations. Typical features include renal tubular injury, self-mutilation, hepatic necrosis, episodic weakness, and seizures.

Abbreviation

Fumarylacetoacetase; FAH

Synonyms

Beta-Diketonase; FAA; FAH; Fumarylacetoacetase; Fumarylacetoacetate Hydrolase

UniProt

P16930

Accession Number

P16930

Expression System

HEK293 Cells

Tag

C-His

Sequence

Ser2-Ser419

Field of Research

Signal Transduction; Cancer; 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

Please refer to the printed manual for detailed information.

Shipping Conditions

This product is provided as lyophilized powder which is shipped with ice packs.

Storage Conditions

Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.

Calculated Molecular Weight

47.4 kDa

Observed Molecular Weight

43 kDa

Species

Human