SPTLC1 Antibody

說明書

中文名稱：

SPTLC1兔多克隆抗體
貨號：

CSB-PA022639GA01HU
規格：

￥3,900
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

O15269
基因名：

SPTLC1
別名：

SPTLC1; LCB1; Serine palmitoyltransferase 1; Long chain base biosynthesis protein 1; LCB 1; Serine-palmitoyl-CoA transferase 1; SPT 1; SPT1
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human SPTLC1
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Serine palmitoyltransferase (SPT). The heterodimer formed with SPTLC2 or SPTLC3 constitutes the catalytic core. The composition of the serine palmitoyltransferase (SPT) complex determines the substrate preference. The SPTLC1-SPTLC2-SPTSSA complex shows a strong preference for C16-CoA substrate, while the SPTLC1-SPTLC3-SPTSSA isozyme uses both C14-CoA and C16-CoA as substrates, with a slight preference for C14-CoA. The SPTLC1-SPTLC2-SPTSSB complex shows a strong preference for C18-CoA substrate, while the SPTLC1-SPTLC3-SPTSSB isozyme displays an ability to use a broader range of acyl-CoAs, without apparent preference. Required for adipocyte cell viability and metabolic homeostasis.
基因功能參考文獻：
1. Therefore, SPT may be an attractive therapeutic anti-cancer drug target for which compound-2 may be a promising new drug. PMID: 28108287
2. Hereditary sensory and autonomic neuropathy type 1 mutations in SPTLC1 have distinct biochemical properties, which allowed for the prediction of the clinical symptoms on the basis of the plasma sphingoid base profile. PMID: 26681808
3. This study describe aberrant morphology of SPTLC1C133W Dorsal Root Ganglia characterized by increased neurite growth, branching, and expression of p-ERM at neuronal growth cones. PMID: 26446223
4. A novel SPTLC2-S384F variant in 2 unrelated HSAN1 families resulted in elevated plasma 1-deoxySL levels. Expression of this mutant in HEK293 cells increased 1-deoxySL formation. The substrate specificity is affected by phosphorylation at this position. PMID: 25567748
5. Therefore, Ser331 in SPTLC1 is a crucial amino acid, which characterizes the Hereditary sensory and autonomic neuropathy type I phenotype PMID: 24247255
6. SPTLC1 mutations cause mitochondrial abnormalities and ER stress in HSN1 cells. PMID: 24673574
7. Endoplasmic reticulum-resident human protein serine palmitoyltransferase long chain-1 (SPTLC1) is phosphorylated at Tyr(164) by the tyrosine kinase ABL. PMID: 23629659
8. The p.CYS133Trp mutation in SPTLC1 is the most common cause of hereditary and autonomic neuropathy in the United Kingdom population. PMID: 22302274
9. SPTLC1 mutations p.S331F and p.A352V result in a reduction of serine palmitoyltransferase activity in vitro and are associated with increased levels of the deoxysphingoid in patients' plasma samples. PMID: 21618344
10. Hereditary sensory neuropathy type 1 is caused by a gain of function mutation in SPTLC1 which causes the accumulation of two neurotoxic sphingolipids PMID: 20097765
11. an increase in transepidermal water loss is an obligatory trigger for the upregulation of serine palmitoyltransferase mRNA expression in human epidermis PMID: 12445191
12. A novel missense mutation in exon 13 of the SPTLC1 gene (c.1160G-->C; p.G387A) in twin sisters with a severe HSN I phenotype is reported. PMID: 15037712
13. SPT1 aggregation preceded cell death in hypoxia and represents the first evidence of acute protein aggregation during hypoxia. PMID: 15467453
14. Transgenic mouse lines that ubiquitously overexpress either wild-type or mutant SPTLC1 represent a novel mouse model of peripheral neuropathy and confirm the link between mutant SPT and neuronal dysfunction. PMID: 16210380
15. Results suggest that functional serine palmitoyltransferase is not a dimer, but a higher organized complex, composed of three distinct subunits (SPTLC1, SPTLC2 and SPTLC3) with a molecular mass of 480 kDa. PMID: 17331073
16. serine palmitoyltransferase and ceramidase are two major ceramide metabolizing enzymes that may have roles in psoriatic epidermis PMID: 17982236
17. SPTLC1 mutational analysis reveals the C133W mutation, a mutation common in British hereditary sensory and autonomic neuropathy type I patients. PMID: 18018475
18. Physical interaction of ABCA1 and SPTLC1 results in reduction of ABCA1 activity and that inhibition of this interaction produces enhanced cholesterol efflux. PMID: 18484747
19. we show that none of the HSAN I mutations interferes with serine palmitoyltransferase complex formation; the G387A mutation is most likely not directly associated with Hereditary sensory neuropathy type 1 PMID: 19132419
20. SPT subunit 1 might be present also in focal adhesions and the nucleus. PMID: 19362163
21. discovery of 2 proteins, ssSPTa and ssSPTb, which each interacts with both hLCB1 and hLCB2, suggesting that there are 4 distinct human SPT isozymes. PMID: 19416851
22. Cell polarity factor Par3 binds SPTLC1 and modulates monocyte serine palmitoyltransferase activity and chemotaxis PMID: 19592499
23. Adult-onset hereditary sensory and autonomic neuropathy type (HSAN)I is the hypothetical result of a gain-of-function mutation in SPTLC1 that leads to accumulation of a toxic metabolite. PMID: 19923297
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相關疾病：

Neuropathy, hereditary sensory and autonomic, 1A (HSAN1A)
亞細胞定位：

Endoplasmic reticulum membrane; Single-pass membrane protein.
蛋白家族：

Class-II pyridoxal-phosphate-dependent aminotransferase family
組織特異性：

Widely expressed. Not detected in small intestine.
數據庫鏈接：

HGNC: 11277

OMIM: 162400

KEGG: hsa:10558

STRING: 9606.ENSP00000262554

UniGene: Hs.90458

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