ACSF3 Antibody (PACO46578)
제품 코드를 사용하여 Assay Genie 메인 사이트를 통해 주문합니다.
주문- SKU:
- PACO46578
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | ACSF3 Antibody (PACO46578) |
Antibody SKU: | PACO46578 |
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 Acyl-CoA synthetase family member 3, mitochondrial protein (365-484AA) |
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 ovarian cancer using PACO46578 at dilution of 1:100. |
![]() | Immunohistochemistry of paraffin-embedded human prostate cancer using PACO46578 at dilution of 1:100. |
Background: | Catalyzes the initial reaction in intramitochondrial fatty acid, synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. May have some preference toward very-long-chain substrates. |
Synonyms: | Acyl-CoA synthetase family member 3, mitochondrial (EC 6.2.1), ACSF3 |
UniProt Protein Function: | ACSF3: Catalyzes the initial reaction in intramitochondrial fatty acid synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. May have some preference toward very-long-chain substrates. Defects in ACSF3 are the cause of combined malonic and methylmalonic aciduria (CMAMMA). A metabolic disease characterized by malonic and methylmalonic aciduria, with urinary excretion of much larger amounts of methylmalonic acid than malonic acid, in the presence of normal malonyl-CoA decarboxylase activity. Clinical features include coma, ketoacidosis, hypoglycemia, failure to thrive, microcephaly, dystonia, axial hypotonia and/or developmental delay, and neurologic manifestations including seizures, psychiatric disease and/or cognitive decline. Belongs to the ATP-dependent AMP-binding enzyme family. |
UniProt Protein Details: | Protein type:Ligase; EC 6.2.1.-; EC 6.-.-.- Chromosomal Location of Human Ortholog: 16q24.3 Cellular Component: mitochondrion Molecular Function:acid-thiol ligase activity; ATP binding Biological Process: fatty acid metabolic process; fatty acid biosynthetic process Disease: Combined Malonic And Methylmalonic Aciduria |
NCBI Summary: | This gene encodes a member of the acyl-CoA synthetase family of enzymes that activate fatty acids by catalyzing the formation of a thioester linkage between fatty acids and coenzyme A. The encoded protein is localized to mitochondria, has high specificity for malonate and methylmalonate and possesses malonyl-CoA synthetase activity. Mutations in this gene are a cause of combined malonic and methylmalonic aciduria. Alternatively spliced transcript variants have been observed for this gene. [provided by RefSeq, Sep 2013] |
UniProt Code: | Q4G176 |
NCBI GenInfo Identifier: | 187761345 |
NCBI Gene ID: | 197322 |
NCBI Accession: | NP_001120686.1 |
UniProt Secondary Accession: | Q4G176,Q6INA0, Q8N2F7, A8K4J8, C9JQL6, |
UniProt Related Accession: | Q4G176 |
Molecular Weight: | 64,130 Da |
NCBI Full Name: | acyl-CoA synthetase family member 3, mitochondrial isoform 1 |
NCBI Synonym Full Names: | acyl-CoA synthetase family member 3 |
NCBI Official Symbol: | ACSF3 |
NCBI Protein Information: | acyl-CoA synthetase family member 3, mitochondrial; malonyl-CoA synthetase |
UniProt Protein Name: | Acyl-CoA synthetase family member 3, mitochondrial |
Protein Family: | Acyl-CoA synthetase family |
UniProt Gene Name: | ACSF3 |
UniProt Entry Name: | ACSF3_HUMAN |
Antibodies |
Anti-ACSF3 Antibody (CAB13769) |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
Recommended Products |
Anti-FITC Goat Anti-Rabbit IgG (H+L) Antibody (CABS011) |
Anti-HRP-conjugated Beta Actin Antibody (CABC028) |