AGPS Rabbit pAb
- 产品详情
- 实验流程
Application
| 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] |
| 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 |
| Storage | Store 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.
| 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
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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