丝氨酸棕榈酰转移酶2抗体
规格：1mg/1ml
英文名： SPTLC2
别名： Serine Palmitoyltransferase; SPTLC-2; LCB 2; LCB2; LCB2a; Long chain base biosynthesis protein 2; Long chain base biosynthesis protein 2a; Serine palmitoyl CoA transferase 2; Serine palmitoyltransfera
分子量： 62kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human SPTLC2
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse,
细胞定位：细胞浆 细胞膜
丝氨酸棕榈酰转移酶2抗体产品介绍：background: This gene encodes a long chain base subunit of serine palmitoyltransferase. Serine palmitoyltransferase, which consists of two different subunits, is the key enzyme in sphingolipid biosynthesis. It catalyzes the pyridoxal-5-prime-phosphate-dependent condensation of L-serine and palmitoyl-CoA to 3-oxosphinganine. Mutations in this gene were identified in patients with hereditary sensory neuropathy type I. [provided by RefSeq, Mar 2011]. Function: Serine palmitoyltransferase (SPT). The heterodimer formed with LCB1/SPTLC1 constitutes the catalytic core. The composition of the serine palmitoyltransferase (SPT) complex determines the substrate preference. The SPTLC1-SPTLC2-SSSPTA complex shows a strong preference for C16-CoA substrate, while the SPTLC1-SPTLC2-SSSPTB complex displays a preference for C18-CoA substrate. Subunit: eterodimer with SPTLC1. Component of the serine palmitoyltransferase (SPT) complex, composed of LCB1/SPTLC1, LCB2 (SPTLC2 or SPTLC3) and ssPT (C14orf147/SSSPTA and C3orf57/SSSPTB). Subcellular Location: Endoplasmic reticulum membrane; Single-pass membrane protein. Tissue Specificity: Widely expressed. DISEASE: Defects in SPTLC2 are the cause of hereditary丝氨酸棕榈酰转移酶2抗体 sensory and autonomic neuropathy type 1C (HSAN1C) [MIM:613640]. It is a form of hereditary sensory and autonomic neuropathy, a genetically and clinically heterogeneous group of disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and by prominent sensory abnormalities with a variable degree of motor and autonomic dysfunction. The neurological phenotype is often complicated by severe infections, osteomyelitis, and amputations. HSAN1C symptoms include loss of touch and vibration in the feet, dysesthesia and severe panmodal sensory loss in the upper and lower limbs, distal lower limb sensory loss with ulceration and osteomyelitis, and distal muscle weakness. Similarity: 丝氨酸棕榈酰转移酶2抗体Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. Database links: Entrez Gene: 9517 Human Entrez Gene: 20773 Mouse Entrez Gene: SPTLC2 Pig Entrez Gene: 366697 Rat Entrez Gene: 553646 Zebrafish Omim: 605713 Human SwissProt: O15270 Human SwissProt: P97363 Mouse Unigene: 435661 Human Unigene: 565 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
丝氨酸棕榈酰转移酶2抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 心血管 细胞生物 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid