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AGPS Rabbit pAb

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
IHC-P, IHC-F, IF
Primary Accession O00116
Other Accession O00116
Host Rabbit
Clonality Polyclonal
Calculated MW 72912 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human AGPS/Alkyl-DHAP synthase
Epitope Specificity 31-130/658
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY Belongs to the FAD-binding oxidoreductase/transferase type 4 family. Contains 1 FAD-binding PCMH-type domain.
SUBUNIT Homodimer.
DISEASE Defects in AGPS are the cause of rhizomelic chondrodysplasia punctata type 3 (RCDP3) [MIM:600121]. RCDP3 is characterized by rhizomelic shortening of femur and humerus, vertebral disorders, cataract, cutaneous lesions and severe mental retardation.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions This gene is a member of the FAD-binding oxidoreductase/transferase type 4 family. It encodes a protein that catalyzes the second step of ether lipid biosynthesis in which acyl-dihydroxyacetonephosphate (DHAP) is converted to alkyl-DHAP by the addition of a long chain alcohol and the removal of a long-chain acid anion. The protein is localized to the inner aspect of the peroxisomal membrane and requires FAD as a cofactor. Mutations in this gene have been associated with rhizomelic chondrodysplasia punctata, type 3 and Zellweger syndrome. [provided by RefSeq, Jul 2008]
Additional Information
Gene ID 8540
Other Names ADAP-S; ADAS; ADHAPS; ADPS; ALDHPSY; RCDP3; 5832437L22; 9930035G10Rik; Adaps; bs2; Ads; ADAS_HUMAN; AGPS; Alkyl-DHAP synthase; Aging-associated gene 5 protein; Alkylglycerone-phosphate synthase; 2.5.1.26; ADAS_MOUSE; ADAS_RAT; alkylglycerone phosphate synthase; Alkyldihydroxyacetonephosphate synthase, peroxisomal
Dilution IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name AGPS
Function Catalyzes the exchange of the acyl chain in acyl- dihydroxyacetonephosphate (acyl-DHAP) for a long chain fatty alcohol, yielding the first ether linked intermediate, i.e. alkyl- dihydroxyacetonephosphate (alkyl-DHAP), in the pathway of ether lipid biosynthesis.
Cellular Location Peroxisome membrane {ECO:0000250|UniProtKB:P97275}. Peroxisome {ECO:0000250|UniProtKB:P97275}
Research Areas
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