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Anti-SDHAF1 Antibody

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产品名称: Anti-SDHAF1 Antibody
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简单介绍

Anti-SDHAF1 Antibody


Anti-SDHAF1 Antibody  的详细介绍
Name: Anti-SDHAF1 Antibody
See all SDHAF1 primary antibodies
Description: Goat polyclonal antibody to SDHAF1.
Applications: ELISA, WB
Reactivity: Human
Immunogen: Synthetic peptide corresponding to Human SDHAF1 (C terminal).
Sequence: C-HDSTGAPETRPDGR
Host: Goat
Clonality: Polyclonal
Isotype: IgG
Conjugate: Unconjugated
Purification: Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Concentration: 100 µg at 0.5 mg/ml.
Product Form: Liquid
Formulation: Supplied in Tris Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 0.5% BSA.
Storage: Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Function: 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.
Tissue Specificity: Ubiquitously expressed.
Involvement in Disease: Mitochondrial complex II deficiency: 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.
Sequence Similarities: Belongs to the complex I LYR family. SDHAF1 subfamily.
Cellular Location: Mitochondrion matrix.
Database Links:
  • Entrez Gene: 644096 Human
  • Omim: 612848 Human
  • SwissProt: A6NFY7 Human
  • Unigene: 356460 Human
    		•
    Synonyms:
  • LYR motif containing 8 Antibody
  • LYR motif-containing protein 8 Antibody
  • LYRM8 Antibody
  • mitochondrial Antibody
  • SDH assembly factor 1 Antibody
  • SDHAF1 Antibody
  • SDHF1_HUMAN Antibody
  • Succinate dehydrogenase assembly factor 1 Antibody
  • Succinate dehydrogenase assembly factor 1 mitochondrial Antibody
  • Succinate dehydrogenase complex assembly factor 1 Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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