Rabbit anti-Human SDHAF1 Polyclonal Antibody | anti-SDHAF1 antibody

SDHAF1, ID (SDHAF1, Succinate Dehydrogenase Assembly Factor 1, Mitochondrial, LYR Motif-containing Protein 8) (Azide free) (HRP)

Cat. Num. AAA6345628

• Gene Names: SDHAF1; LYRM8
• Reactivity: Human
• Applications: Immunohistochemistry, ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: SDHAF1; Polyclonal Antibody; ID (SDHAF1; Succinate Dehydrogenase Assembly Factor 1; Mitochondrial; LYR Motif-containing Protein 8) (Azide free) (HRP); mitochondrial; LYR Motif-containing Protein 8; anti-SDHAF1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish Peroxidase (HRP).

• Applicable Applications for anti-SDHAF1 antibody: Immunohistochemistry (IHC), ELISA (EIA), Western Blot (WB)
• Application Notes: IHC: 1:50-100; ELISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: SDHAF1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 41-70 amino acids from the Central region of human SDHAF1.
• Conjugate: HRP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(SDHAF1 Antibody (Center) western blot analysis in ZR-75-1 cell line lysates (35ug/lane).This demonstrates the SDHAF1 antibody detected the SDHAF1 protein (arrow).)

Immunohistochemistry (IHC)

(SDHAF1 Antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human prostate carcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the SDHAF1 Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.)

Related Product Information for anti-SDHAF1 antibody

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).

Product Categories/Family for anti-SDHAF1 antibody

Antibodies; Mitochondrial Proteins

References

Ghezzi, D., et al. Nat. Genet. (2009). In press: Hoffmann, T.W., et al. Transplant. Proc. 41(2):654-656(2009).

NCBI and Uniprot Product Information

• NCBI GI #: 111038124
• NCBI GeneID: 644096
• NCBI Accession #: NP_001036096.1
• NCBI GenBank Nucleotide #: NM_001042631.2
• UniProt Accession #: A6NFY7; B2RPM7
• Molecular Weight: 12,806 Da
• NCBI Official Full Name: succinate dehydrogenase assembly factor 1, mitochondrial
• NCBI Official 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 Gene Name: SDHAF1
• UniProt Synonym Gene Names: SDHAF1
• UniProt Entry Name: SDHF1_HUMAN

Uniprot Description

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: 19q13.12

Cellular Component: mitochondrion

Disease: Mitochondrial Complex Ii Deficiency