Metabolism Antibodies 1
Anti-ACAT1 Antibody (CAB13273)
- SKU:
- CAB13273
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
Antibody Name: | Anti-ACAT1 Antibody |
Antibody SKU: | CAB13273 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB IHC IF |
Reactivity: | Human, Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-145 of human ACAT1 (NP_000010.1). |
Application: | WB IHC IF |
Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
Reactivity: | Human, Mouse, Rat |
Positive Samples: | SW620, Raji, H460, HepG2, Mouse liver, Mouse testis, Mouse small intestine |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-145 of human ACAT1 (NP_000010.1). |
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: | MAVL AALL RSGA RSRS PLLR RLVQ EIRY VERS YVSK PTLK EVVI VSAT RTPI GSFL GSLS LLPA TKLG SIAI QGAI EKAG IPKE EVKE AYMG NVLQ GGEG QAPT RQAV LGAG LPIS TPCT TINK VCAS GMKA IMMA SQSL MCGH Q |
Gene ID: | 38 |
Uniprot: | P24752 |
Cellular Location: | Mitochondrion |
Calculated MW: | 42kDa |
Observed MW: | 42kDa |
Synonyms: | ACAT1, ACAT, MAT, T2, THIL |
Background: | This gene encodes a mitochondrially localized enzyme that catalyzes the reversible formation of acetoacetyl-CoA from two molecules of acetyl-CoA. Defects in this gene are associated with 3-ketothiolase deficiency, an inborn error of isoleucine catabolism characterized by urinary excretion of 2-methyl-3-hydroxybutyric acid, 2-methylacetoacetic acid, tiglylglycine, and butanone. |
UniProt Protein Function: | ACAT1: Plays a major role in ketone body metabolism. Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD); also known as alpha- methylacetoaceticaciduria. 3KTD is an inborn error of isoleucine catabolism characterized by intermittent ketoacidotic attacks associated with unconsciousness. Some patients die during an attack or are mentally retarded. Urinary excretion of 2-methyl-3- hydroxybutyric acid, 2-methylacetoacetic acid, triglylglycine, butanone is increased. It seems likely that the severity of this disease correlates better with the environmental or acquired factors than with the ACAT1 genotype. Belongs to the thiolase family. |
UniProt Protein Details: | Protein type:Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - fatty acid; Acetyltransferase; Lipid Metabolism - synthesis and degradation of ketone bodies; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - tryptophan; EC 2.3.1.9; Amino Acid Metabolism - lysine degradation; Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - butanoate; Mitochondrial Chromosomal Location of Human Ortholog: 11q22.3 Cellular Component: mitochondrial matrix; mitochondrion Molecular Function:acetyl-CoA C-acetyltransferase activity Biological Process: branched chain family amino acid catabolic process; ketone body biosynthetic process; ketone body catabolic process Disease: Alpha-methylacetoacetic Aciduria |
NCBI Summary: | This gene encodes a mitochondrially localized enzyme that catalyzes the reversible formation of acetoacetyl-CoA from two molecules of acetyl-CoA. Defects in this gene are associated with 3-ketothiolase deficiency, an inborn error of isoleucine catabolism characterized by urinary excretion of 2-methyl-3-hydroxybutyric acid, 2-methylacetoacetic acid, tiglylglycine, and butanone. [provided by RefSeq, Feb 2009] |
UniProt Code: | P24752 |
NCBI GenInfo Identifier: | 135755 |
NCBI Gene ID: | 38 |
NCBI Accession: | P24752.1 |
UniProt Secondary Accession: | P24752,Q96FG8, B2R6H1, G3XAB4, |
UniProt Related Accession: | P24752 |
Molecular Weight: | 17,175 Da |
NCBI Full Name: | Acetyl-CoA acetyltransferase, mitochondrial |
NCBI Synonym Full Names: | acetyl-CoA acetyltransferase 1 |
NCBI Official Symbol: | ACAT1 |
NCBI Official Synonym Symbols: | T2; MAT; ACAT; THIL |
NCBI Protein Information: | acetyl-CoA acetyltransferase, mitochondrial |
UniProt Protein Name: | Acetyl-CoA acetyltransferase, mitochondrial |
UniProt Synonym Protein Names: | Acetoacetyl-CoA thiolase; T2 |
UniProt Gene Name: | ACAT1 |
UniProt Entry Name: | THIL_HUMAN |