Recombinant Human SBDS protein (His tag N-Terminus)

Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

SBDS also known as Shwachman-Bodian-Diamond syndrome protein participates in ribosome biogenesis. It has a molecular weight of approximately 29 kDa. SBDS is expressed in a variety of tissues with notable expression in the bone marrow and pancreas areas that require active ribosome synthesis. The protein works to assist in the assembly and maturation of the 60S ribosomal subunit associating closely with other ribosomal proteins and factors needed for proper cellular function.

Biological function summary

SBDS plays an essential role in the maturation of ribosomes and is part of the ribosomal large subunit assembly complex. It interacts with proteins such as EFL1 and Nucleophosmin highlighting its involvement in vital cellular processes. The protein contributes to protein synthesis machinery ensuring that ribosomal subunits are correctly formed and functional. SBDS is important for maintaining cellular homeostasis through its engagement in ribosome assembly.

Pathways

SBDS is involved in ribosome biogenesis a fundamental pathway for cell growth and division. This process is closely connected with proteins like eIF6 which cooperates with SBDS during the final stages of ribosomal subunit maturation. Additionally SBDS aligns with pathways related to cellular stress responses given its role in ensuring the integrity and function of ribosomes under conditions of cellular stress. These interactions demonstrate SBDS's importance in overarching cellular pathways that sustain life.

Mutations in the SBDS gene cause Shwachman-Diamond syndrome a rare congenital disorder that affects bone marrow function and pancreatic enzymes. The syndrome often results in neutropenia and pancreatic insufficiency manifesting through the disruption of ribosome biogenesis. Moreover research suggests SBDS might interact with proteins involved in other ribosomopathies connecting it indirectly to a spectrum of related disorders. Understanding SBDS’s role in these contexts provides insight into therapeutic targets and interventions for diseases involving ribosomal dysfunction.