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Anti-SDHAF1 Antibody

品牌：Antibodies

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規(guī)格：100μg

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Anti-SDHAF1 Antibody

商品詳情參考文獻(xiàn) 相關(guān)資料

Overview

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.

Target

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.