Recombinant Human PCCA, N-His

Description of Recombinant Human PCCA, N-His

Introduction

Recombinant Human PCCA, N-His is a protein that has been genetically engineered in the laboratory using recombinant DNA technology. This protein plays a crucial role in the metabolism of amino acids and is a potential drug target for various metabolic disorders.

Structure of Recombinant Human PCCA, N-His

The recombinant form of PCCA, N-His is identical in structure to the native human protein. It is composed of 1091 amino acids and has a molecular weight of approximately 120 kDa. The protein consists of two subunits, alpha and beta, which are encoded by the PCCA and PCCB genes, respectively.

The alpha subunit contains the catalytic domain, while the beta subunit is responsible for binding to other proteins and regulatory functions. The two subunits come together to form a heterodimer, which is the active form of the protein.

Activity of this protein

Recombinant Human PCCA, N-His is a biologically active protein that plays a crucial role in the metabolism of amino acids. It is a key component of the enzyme propionyl-CoA carboxylase (PCC), which is responsible for converting propionyl-CoA to methylmalonyl-CoA.

This reaction is essential for the breakdown of certain amino acids, fatty acids, and cholesterol. Any dysfunction in the PCC enzyme can lead to a buildup of toxic metabolites, resulting in metabolic disorders such as propionic acidemia and methylmalonic acidemia.

The recombinant form of PCCA, N-His is highly specific and efficient in its catalytic activity, making it a potential therapeutic target for the treatment of these disorders.

Application of Recombinant Human PCCA, N-His

Recombinant Human PCCA, N-His has various applications in the field of medicine. Its primary use is in the treatment of metabolic disorders caused by PCC enzyme deficiency, such as propionic acidemia and methylmalonic acidemia.

Currently, there are no FDA-approved drugs that target PCC enzyme deficiency. However, recombinant PCCA, N-His shows promising results in pre-clinical and clinical trials for the treatment of these disorders. It has been shown to improve metabolic control and reduce the levels of toxic metabolites in patients with propionic acidemia and methylmalonic acidemia.

In addition to its therapeutic applications, recombinant PCCA, N-His also has potential uses in diagnostic testing for PCC enzyme deficiency. It can be used to measure the activity of the PCC enzyme in patient samples, aiding in the diagnosis and monitoring of these disorders.

Conclusion

Recombinant Human PCCA, N-His is a biologically active protein that plays a crucial role in the metabolism of amino acids. Its specific and efficient catalytic activity makes it a potential drug target for the treatment of metabolic disorders caused by PCC enzyme deficiency. Its applications in therapy and diagnostics make it a valuable tool in the field of medicine.