
Anti-SPTLC1 Antibody
Price on request
| SKU | Price | Quantity |
|---|---|---|
| A40418-100 | Price on request |
Description
This description has not been verified against the manufacturer's datasheet. Check the datasheet before you order, or ask us to confirm a specification. Ask us to confirm
Serine palmitoyltransferase long chain base subunit 1 (SPTLC1) is the catalytic core of the ER-localized enzyme complex that condenses L-serine with palmitoyl-CoA in the first committed step of sphingolipid biosynthesis. The rabbit polyclonal Anti-SPTLC1 reagent distributed by Antibodies.com is supplied as a 100 µL unconjugated aliquot validated for Western blot and ELISA workflows, with confirmed reactivity against human, mouse, and rat orthologues.
The target sits at a notable intersection of basic lipid metabolism and human disease. Dominant missense mutations in SPTLC1, including C133W, C133Y, and V144D, shift substrate specificity toward L-alanine and L-glycine, producing deoxysphingoid bases that cause hereditary sensory neuropathy type 1 (HSAN1). More recently, recessive SPTLC1 loss-of-function alleles have been identified in juvenile amyotrophic lateral sclerosis, broadening the clinical relevance of accurate protein-level detection in patient-derived fibroblasts, lymphoblasts, and iPSC-derived motor neurons.
Beyond hereditary neuropathy research, the antibody supports studies of ceramide-dependent signaling in metabolic disease, neuroinflammation, and membrane biology. Common applications include confirming knockdown or overexpression in HEK293 and HeLa cell lines, monitoring ER stress responses, and tracking SPTLC1 distribution in tissue lysates from brain, liver, and dorsal root ganglia. Suggested Western blot dilutions range from 1:500 to 1:2000 depending on antigen abundance, and ELISA protocols can be adapted to standard microplate formats. For short-term use, store refrigerated at 4 °C; for longer intervals, aliquot and freeze at -20 °C and limit freeze-thaw cycles to preserve antibody performance.
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