The SDHAF1 Antibody (PAC054102) is a polyclonal antibody designed for research involving SDHAF1, a protein involved in the assembly and function of succinate dehydrogenase (SDH), a key enzyme in the citric acid cycle. The antibody, produced in rabbits, is highly specific to human samples and has been validated for use in Western blot applications. It binds specifically to the SDHAF1 protein, allowing for the detection and analysis of SDHAF1 in various cell types.SDHAF1 is a crucial component of the SDH complex, which plays a critical role in cellular energy production and metabolism. Mutations in SDHAF1 have been linked to various diseases, including cancer and neurodegenerative disorders.
Research into the function and regulation of SDHAF1 is essential for understanding its role in these conditions and may lead to potential therapeutic interventions targeting the SDH pathway.The SDHAF1 Antibody is a valuable tool for scientists studying SDHAF1 and its related pathways in both normal and disease states. Its high reactivity and specificity make it an ideal choice for research in biochemistry, molecular biology, and medicine, providing valuable insights into the role of SDHAF1 in cellular metabolism and disease development.
Antibody Name:
SDHAF1 Antibody (PACO54102)
Antibody SKU:
PACO54102
Size:
50ug
Host Species:
Rabbit
Tested Applications:
ELISA, IHC
Recommended Dilutions:
ELISA:1:2000-1:10000, IHC:1:20-1:200
Species Reactivity:
Human
Immunogen:
Recombinant Human Succinate dehydrogenase assembly factor 1, mitochondrial protein (78-115AA)
Immunohistochemistry of paraffin-embedded human kidney tissue using PACO54102 at dilution of 1:100.
Immunohistochemistry of paraffin-embedded human placenta tissue using PACO54102 at dilution of 1:100.
Background:
Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid, (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF3.
SDHAF1: Plays an essential role in succinate dehydrogenase complex (SDH) assembly, a complex involved in complex II of the mitochondrial electron transport chain. Probably acts by participating in mitochondrial biosynthesis of iron-sulfur centers for complex II (Probable). Defects in SDHAF1 are a cause of mitochondrial complex II deficiency (MT-C2D); also known as SDH-defective infantile leukoencephalopathy. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome. Belongs to the complex I LYR family. SDHAF1 subfamily.Chromosomal Location of Human Ortholog: 19q13.12Cellular Component: mitochondrion; mitochondrial matrixBiological Process: iron-sulfur cluster assemblyDisease: Mitochondrial Complex Ii Deficiency
UniProt Protein Details:
NCBI Summary:
The succinate dehydrogenase (SDH) complex (or complex II) of the mitochondrial respiratory chain is composed of 4 individual subunits. The protein encoded by this gene resides in the mitochondria, and is essential for SDH assembly, but does not physically associate with the complex in vivo. Mutations in this gene are associated with SDH-defective infantile leukoencephalopathy (mitochondrial complex II deficiency).[provided by RefSeq, Mar 2010]
