Metabolism Antibodies 1

Anti-ACSF3 Antibody (CAB13769)

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SKU:
CAB13769
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-ACSF3 Antibody
Antibody SKU:CAB13769
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 270-450 of human ACSF3 (NP_001230208.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:U-87MG, 293T, Mouse liver, Mouse heart, Mouse kidney, Rat brain
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 270-450 of human ACSF3 (NP_001230208.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:FSPQ QVWE KFLS SETP RINV FMAV PTIY TKLM EYYD RHFT QPHA QDFL RAVC EEKI RLMV SGSA ALPL PVLE KWKN ITGH TLLE RYGM TEIG MALS GPLT TAVR LPGS VGTP LPGV QVRI VSEN PQRE ACSY TIHA EGDE RGTK VTPG FEEK EGEL LVRG PSVF REYW NKPE ETKS AFTL D
Gene ID:197322
Uniprot:Q4G176
Cellular Location:Mitochondrion
Calculated MW:64kDa
Observed MW:64kDa
Synonyms:ACSF3
Background: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.
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
Anti-ACSF3 Antibody (CAB13769)Western blot analysis of extracts of various cell lines, using ACSF3 antibody (CAB13769) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 60s.
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