Human SDHAF1 Recombinant Protein (RPPB4605)

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SKU:
RPPB4605
Product Type:
Recombinant Protein
Species:
Human
Uniprot:
A6NFY7

Description

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Product Name:Human SDHAF1 Recombinant Protein
Product Code:RPPB4605
Size:10µg
Species:Human
Target:SDHAF1
Synonyms:Succinate Dehydrogenase Complex Assembly Factor 1, LYRMotif-Containing Protein 8, LYR MotifContaining 8, SDH Assembly Factor 1, LYRM8, Succinate Dehydrogenase AssemblyFactor 1, Mitochondrial, Succinate dehydrogenase assembly factor 1,mitochondrial.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:SDHAF1 protein solution (0.25mg/ml) containing Phosphatebuffered saline (pH7.4), 30% glycerol, 2mM DTT and 0.1mM PMSF.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 85.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSMSRHSRL QRQVLSLYRD LLRAGRGKPG AEARVRAEFR QHAGLPRSDVLRIEYLYRRG RRQLQLLRSG HATAMGAFVR PRAPTGEPGG VGSQPDDGDS PRNPHDSTGA PETRPDGR

Succinate dehydrogenase assembly factor 1 (SDHAF1) is a member of the complexI LYR family. SDHAF1 plays an important role in succinate dehydrogenase complex(SDH) assembly, a complex which is involved in complex II of the mitochondrialelectron transport chain. SDHAF1 functions through taking part in mitochondrialbiosynthesis of iron-sulfur centers for complex II.

SDHAF1 Human Recombinant produced in E.Coli is a single,non-glycosylated polypeptide chain containing 138 amino acids (1-115 a.a) andhaving a molecular mass of 15.2kDa.SDHAF1 is fused to a 23 amino acid His-tag at N-terminus& purified by proprietary chromatographic techniques.

UniProt Protein Function: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]
UniProt Code:A6NFY7
NCBI GenInfo Identifier:111038124
NCBI Gene ID:644096
NCBI Accession:NP_001036096.1
UniProt Secondary Accession:A6NFY7,B2RPM7
UniProt Related Accession:A6NFY7
Molecular Weight:12806
NCBI Full Name:succinate dehydrogenase assembly factor 1, mitochondrial
NCBI Synonym Full Names:succinate dehydrogenase complex assembly factor 1
NCBI Official Symbol:SDHAF1��
NCBI Official Synonym Symbols:LYRM8��
NCBI Protein Information:succinate dehydrogenase assembly factor 1, mitochondrial
UniProt Protein Name:Succinate dehydrogenase assembly factor 1, mitochondrial
UniProt Synonym Protein Names:LYR motif-containing protein 8
Protein Family:
UniProt Gene Name:SDHAF1��
UniProt Entry Name:SDHF1_HUMAN
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