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Rabbit Anti-ACAT1/PE Conjugated antibody
Rabbit Anti-ACAT1/PE Conjugated antibody
ACAT 1; ACAT; Acetoacetyl CoA thiolase; Acetyl CoA acetyltransferase, mitochondrial; Acetyl Coenzyme A acetyltransferase 1; MAT; RATACAL; T2; THIL; THIL_HUMAN; Acetyl-CoA acetyltransferase, mitochondrial; Acetoacetyl-CoA thiolase; T2.
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Details

Product Name Anti-ACAT1/PE
Chinese Name PE标记的乙酰辅酶A酰基转移酶1抗体
Alias ACAT 1; ACAT; Acetoacetyl CoA thiolase; Acetyl CoA acetyltransferase, mitochondrial; Acetyl Coenzyme A acetyltransferase 1; MAT; RATACAL; T2; THIL; THIL_HUMAN; Acetyl-CoA acetyltransferase, mitochondrial; Acetoacetyl-CoA thiolase; T2.  
Research Area Tumour  Cell biology  immunology  transcriptional regulatory factor  Mitochondrion  
Immunogen Species Rabbit
Clonality Polyclonal
React Species (predicted: Human, Mouse, Rat, Dog, Rabbit, )
Applications IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
Molecular weight 47kDa
Form Lyophilized or Liquid
Concentration 1mg/ml
immunogen KLH conjugated synthetic peptide derived from human ACAT1
Lsotype IgG
Purification affinity purified by Protein A
Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail 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. [provided by RefSeq, Feb 2009].

Function:
Plays a major role in ketone body metabolism.

Subunit:
Homotetramer.

Subcellular Location:
Mitochondrion.

DISEASE:
Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD) [MIM:203750]; 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.

Similarity:
Belongs to the thiolase family.

Database links:

Entrez Gene: 38 Human

Entrez Gene: 110446 Mouse

Entrez Gene: 25014 Rat

Omim: 607809 Human

SwissProt: P24752 Human

SwissProt: Q8QZT1 Mouse

SwissProt: P17764 Rat

Unigene: 232375 Human

Unigene: 293233 Mouse

Unigene: 4054 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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