89-7765-38 ［取扱停止］Recombinant Human ATL1/SPG3A/Atlastin-1 Protein (GST Tag) 20ug PKSH031549
[Elabscience]
特徴
- 背景：Atlastin-1, also known as Spastic paraplegia 3 protein A, Guanine nucleotide-binding protein 3, GTP-binding protein 3, GBP3, ATL1 and SPG3A, is a multi-pass membrane protein which belongs to the GBP family and atlastin subfamily. ATL1 / SPG3A is expressed predominantly in the adult and fetal central nervous system. Expression of ATL1 / SPG3A in adult brain is at least 50-fold higher than in other tissues. ATL1 / SPG3A is detected predominantly in pyramidal neurons in the cerebral cortex and the hippocampus of the brain. ATL1 / SPG3A is also expressed in upper and lower motor neurons (at protein level). A distinguishing feature of ATL1 / SPG3A is its frequent early onset, raising the possibility that developmental abnormalities may be involved in its pathogenesis. Missense SPG3A mutant atlastin-1 proteins have impaired GTPase activity and may act in a dominant-negative, loss-of-function manner by forming mixed oligomers with wild-type atlastin-1. Defects in ATL1 / SPG3A are the cause of spastic paraplegia autosomal dominant type 3 (SPG3), also known as Strumpell-Lorrain syndrome. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs.
仕様
- サイズ：20ug
- 種由来：Human
- 発現宿主：Baculovirus-Insect Cells
- 純度：> 80 % as determined by reducing SDS-PAGE.
- エンドトキシン濃度：< 1.0 EU per ug as determined by the LAL method.
- アクセッションＮｏ．：NP_056999.2
- タグ種類：N-GST
- アミノ酸配列：Met 1-Thr 447
- 遺伝子構造：A DNA sequence encoding the human SPG3A (NP_056999.2) (Met 1-Thr 447) was fused with the GST tag at the N-terminus.
- 別名（遺伝子名）：AD-FSP;atlastin1;FSP1;GBP3;HSN1D;SPG3;SPG3A
- 分子量：77 kDa
- 組成：Lyophilized from sterile 50mM Tris, 100mM NaCl, 0.5mM PMSF, 0.5mM EDTA, 0.5mM GSH, pH 8.0
- 保存条件：Lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months.
- メーカーマニュアルリンク：https://www.elabscience.com/viewpdf-38003-Elabscience-PKSH031549.pdf
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商品のバリエーション （サイズ違い・スペック違い・オプション品など）
| 商品イメージ | アズワン品番 商品名 |
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89-7765-38
Recombinant Human ATL1/SPG3A/Atlastin-1 Protein (GST Tag) 20ug
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89-7765-39
Recombinant Human ATL1/SPG3A/Atlastin-1 Protein (GST Tag) 100ug
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