SDHAF1 Antibody (PACO54102)
- SKU:
- PACO54102
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- IHC
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| 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) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | 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. |
| Synonyms: | Succinate dehydrogenase assembly factor 1, mitochondrial (SDH assembly factor 1) (SDHAF1) (LYR motif-containing protein 8), SDHAF1, LYRM8 |
| 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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