RB1 Human
(a) Analysis by RP-HPLC.
(b) Analysis by SDS-PAGE.
Description
The Retinoblastoma is purified by proprietary chromatographic techniques.
Product Specs
TSEKFQKINQMVCNSDRVLKRSAEGSNPPKPLKKLRFDIEGSDEADGSK
HLPGESKFQQKLAEMTSTRTRMQKQKMNDSMDTSNKEEKHHHHHH.
Product Science Overview
Retinoblastoma associated protein, commonly referred to as pRB, is a crucial tumor suppressor protein encoded by the RB1 gene. This protein plays a significant role in regulating the cell cycle and preventing uncontrolled cell proliferation. The human recombinant form of this protein is produced using recombinant DNA technology, which allows for the expression of the protein in various host systems, such as insect cells .
Retinoblastoma is a rare form of eye cancer that primarily affects young children. It is caused by mutations in the RB1 gene, which lead to the inactivation of the retinoblastoma protein. The RB1 gene harbors a wide spectrum of pathogenic variants, and tumorigenesis begins with mutations that cause RB1 biallelic inactivation, preventing the production of functional pRB proteins . This inactivation disrupts the cell cycle control, leading to uncontrolled cell division and tumor formation.
The retinoblastoma protein (pRB) is a key regulator of the cell cycle. It functions by binding to transcription factors of the E2F family, thereby repressing the transcription of genes essential for cell cycle progression, such as cdc2, cyclin A, and oncogenes like c-Myc and c-Fos . When pRB is phosphorylated, it releases E2F transcription factors, allowing the cell to progress from the G1 phase to the S phase of the cell cycle. This regulation ensures that cells only divide when necessary and helps prevent the development of cancer.
Beyond its role in cell cycle regulation, pRB has several other important functions. It is involved in:
- Apoptosis: pRB can induce programmed cell death in response to DNA damage, preventing the propagation of damaged cells .
- DNA Replication and Repair: pRB participates in the replication and repair of DNA, maintaining genomic stability .
- Cellular Senescence and Differentiation: pRB plays a role in cellular aging and differentiation, contributing to the development of specialized cell types .
- Chromatin Remodeling: pRB modulates chromatin structure, influencing gene expression and cellular responses to environmental signals .
Understanding the molecular mechanisms of pRB and its role in retinoblastoma has significant clinical implications. The identification of RB1 mutations and the development of recombinant pRB have paved the way for improved diagnostic and therapeutic approaches. High-throughput techniques are now essential for credible biomarker identification and patient management . Additionally, the study of pRB has contributed to our broader understanding of tumor suppressor genes and cancer biology.
