P4HB Mouse
Description
P4HB produced in Sf9 Baculovirus cells is a single, glycosylated polypeptide chain containing 498 amino acids (20-509a.a.) and having a molecular mass of 56.1kDa. P4HB is expressed with an 8 amino acid His tag at C-Terminus and purified by proprietary chromatographic techniques.
Product Specs
Product Science Overview
P4HB is a subunit of the prolyl 4-hydroxylase enzyme complex, which is responsible for the hydroxylation of proline residues in preprocollagen. This hydroxylation is essential for the stability and function of collagen, a major structural protein in the extracellular matrix . The enzyme complex typically consists of two alpha and two beta subunits, with the beta subunit being P4HB .
P4HB has four thioredoxin domains (a, b, b’, and a’), with two CGHC active sites in the a and a’ domains. These domains are arranged in a horseshoe shape, which changes conformation between its reduced and oxidized states to facilitate substrate binding and catalysis .
Beyond its role in collagen synthesis, P4HB exhibits several other functions:
- Disulfide Isomerase Activity: It catalyzes the formation, breakage, and rearrangement of disulfide bonds in proteins, which is crucial for protein folding and stability .
- Chaperone Activity: P4HB acts as a chaperone, preventing the aggregation of misfolded proteins in a concentration-dependent manner .
- Thyroid Hormone Binding: It can bind thyroid hormones, influencing their transport and metabolism .
- Nitric Oxide Regulation: P4HB is involved in the influx and efflux of S-nitrosothiol-bound nitric oxide, playing a role in cellular signaling .
- Microsomal Triglyceride Transfer Protein Complex: It functions as a subunit of this complex, which is essential for lipid metabolism .
P4HB has been implicated in various diseases and conditions:
- Neurodegenerative Diseases: Elevated levels of nitrosylated P4HB have been observed in brain tissues of patients with Parkinson’s and Alzheimer’s diseases, as well as in spinal cord tissues of individuals with amyotrophic lateral sclerosis .
- Cancer: Upregulation of P4HB has been noted in glioblastoma multiforme, a type of brain tumor .
- Genetic Disorders: Mutations in the P4hb gene can lead to conditions such as Cole-Carpenter Syndrome, characterized by short stature and intrauterine growth retardation .
Recombinant P4HB from mice is widely used in research to study its various functions and roles in disease. Understanding the mechanisms of P4HB can lead to the development of therapeutic strategies for conditions associated with its dysfunction.
