Recombinant Human AMACR protein (ab161708)
- Datasheet
- References
- Protocols
Description
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Product name
Recombinant Human AMACR protein -
Expression system
Wheat germ -
Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
MALQGISVMELSGLAPGPFCAMVLADFGARVVRVDRPGSRYDVSRLGRGK RSLVLDLKQPRGAAVLRRLCKRSDVLLEPFRRGVMEKLQLGPEILQRENP RLIYARLSGFGQSGSFCRLAGHDINYLALSGGRNSMFKFFSVENSEIESV GSTSRTEHVGWWSTFLYDLQDSRWGIHGCWSNRTPVLRAADQRTWTKV -
Amino acids
1 to 198 -
Tags
GST tag N-Terminus
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Specifications
Our Abpromise guarantee covers the use of ab161708 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
ELISA
Western blot
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Form
Liquid -
Additional notes
This product was previously labelled as AMACR, AMCR.
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Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00
Constituents: 0.31% Glutathione, 0.79% Tris HCl
General Info
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Alternative names
- 2 arylpropionyl CoA epimerase
- 2 methylacyl CoA racemase
- 2-methylacyl-CoA racemase
see all -
Function
Racemization of 2-methyl-branched fatty acid CoA esters. Responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers. -
Pathway
Lipid metabolism; bile acid biosynthesis.
Lipid metabolism; fatty acid metabolism. -
Involvement in disease
Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD) [MIM:614307]. AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. It can be associated with polyneuropathy, retinitis pigmentosa, epilepsy.
Defects in AMACR are the cause of congenital bile acid synthesis defect type 4 (CBAS4) [MIM:214950]; also known as cholestasis, intrahepatic, with defective conversion of trihydroxycoprostanic acid to cholic acid or trihydroxycoprostanic acid in bile. Clinical features include neonatal jaundice, intrahepatic cholestasis, bile duct deficiency and absence of cholic acid from bile. -
Sequence similarities
Belongs to the CaiB/BaiF CoA-transferase family. -
Cellular localization
Peroxisome. Mitochondrion. - Information by UniProt
Images
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SDS-PAGE - Recombinant Human AMACR protein (ab161708)ab161708 on a 12.5% SDS-PAGE stained with Coomassie Blue.
Datasheets and documents
References
ab161708 has not yet been referenced specifically in any publications.
