Recombinant Proteins
Product List
SDHAF1 Human
Succinate Dehydrogenase Complex Assembly Factor 1 Human Recombinant
SDHAF2 Human
Succinate Dehydrogenase Complex Assembly Factor 2 Human Recombinant
SDHAF2 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
Introduction
SDHAF, or Succinate Dehydrogenase Assembly Factor, refers to a group of proteins essential for the assembly and function of the succinate dehydrogenase (SDH) complex, also known as mitochondrial complex II. This complex plays a crucial role in both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain. The primary members of this group include SDHAF1 and SDHAF2 .
Key Biological Properties: SDHAF proteins are mitochondrial proteins involved in the assembly of the SDH complex. They are essential for the proper function of the SDH complex, which is crucial for cellular respiration and energy production .
Expression Patterns: SDHAF1 and SDHAF2 are ubiquitously expressed in various tissues, with higher expression levels observed in metabolically active tissues such as the heart, liver, and muscles .
Tissue Distribution: SDHAF proteins are predominantly localized in the mitochondria, where they interact with other components of the SDH complex to facilitate its assembly and function .
Primary Biological Functions: The primary function of SDHAF proteins is to assist in the assembly and stabilization of the SDH complex. This complex is involved in the oxidation of succinate to fumarate in the TCA cycle and the transfer of electrons to the mitochondrial electron transport chain .
Role in Immune Responses and Pathogen Recognition: While the primary role of SDHAF proteins is in cellular respiration, recent studies suggest that they may also play a role in immune responses and pathogen recognition by modulating cellular metabolism and reactive oxygen species (ROS) production .
Mechanisms with Other Molecules and Cells: SDHAF proteins interact with various components of the SDH complex, including SDHA, SDHB, SDHC, and SDHD subunits. They facilitate the incorporation of cofactors such as flavin adenine dinucleotide (FAD) and iron-sulfur clusters into the SDH complex .
Binding Partners and Downstream Signaling Cascades: SDHAF proteins bind to the catalytic subunits of the SDH complex, promoting their proper folding and assembly. This interaction is crucial for the activation of downstream signaling cascades involved in cellular energy production and metabolism .
Regulatory Mechanisms Controlling Expression and Activity: The expression and activity of SDHAF proteins are regulated at multiple levels, including transcriptional and post-transcriptional mechanisms. Transcription factors such as HIF-1α and NRF2 have been shown to regulate the expression of SDHAF genes in response to cellular metabolic states .
Transcriptional Regulation and Post-Translational Modifications: SDHAF proteins undergo various post-translational modifications, including phosphorylation and ubiquitination, which modulate their stability and activity. These modifications are crucial for the dynamic regulation of the SDH complex in response to cellular metabolic demands .
Biomedical Research: SDHAF proteins are valuable tools in biomedical research for studying mitochondrial function and metabolic disorders. They serve as markers for mitochondrial dysfunction and are used to investigate the molecular mechanisms underlying various diseases .
Diagnostic Tools: Mutations in SDHAF genes are associated with several inherited metabolic disorders, including mitochondrial complex II deficiency and paraganglioma. Genetic testing for SDHAF mutations is used as a diagnostic tool for these conditions .
Therapeutic Strategies: Targeting SDHAF proteins and their interactions with the SDH complex holds potential for developing therapeutic strategies for metabolic disorders and mitochondrial diseases. Modulating SDHAF activity may help restore mitochondrial function and improve cellular energy production .
Role Throughout the Life Cycle: SDHAF proteins play a critical role throughout the life cycle, from development to aging. During development, they are essential for proper mitochondrial function and energy production, which are crucial for cell growth and differentiation .
From Development to Aging and Disease: In aging, the activity of SDHAF proteins and the SDH complex may decline, leading to impaired mitochondrial function and increased susceptibility to metabolic disorders. Mutations in SDHAF genes are also linked to various diseases, including cancer and neurodegenerative disorders .
