货号 | AF2237-SP |
别名 | Cell growth-inhibiting gene 1 protein; ceroid-lipofuscinosis, neuronal 2, late infantile (Jansky-Bielschowsky disease); CLN2; CLN2EC 3.4.14.9; growth-inhibiting protein 1; LINCL; LPIC; lysosomal pepstatin insensitive protease; Lysosomal pepstatin-insensitive protease; MGC21297; TPP1; TPP-1; TPP-I; Tripeptidyl aminopeptidase; tripeptidyl peptidase I; tripeptidyl-peptidase 1; Tripeptidyl-peptidase I |
反应种属 | Human |
应用 | Western Blot(0.1 µg/mL) Immunoprecipitation(25 µg/mL) |
目标/特异性 | Detects human Tripeptidyl‑Peptidase I/TPP1 in direct ELISAs and Western blots. |
使用方法 | Western Blot: 0.1 µg/mL Immunoprecipitation: 25 µg/mL |
来源 | Reconstitute at 0.2 mg/mL in sterile PBS. |
产品组分 |
供应商 | R&D Systems |
Entrez Gene IDs | 1200 (Human) |
纯化方式 | Antigen Affinity-purified |
免疫原 | Mouse myeloma cell line NS0-derived recombinant human Tripeptidyl‑Peptidase I/TPP1 Ser20-Pro563 Accession # O14773 |
生物活性 | Human |
标记 | Unconjugated |
溶解方法 | Reconstitute at 0.2 mg/mL in sterile PBS. |
背景 | Tripeptidyl-peptidase I (TPP1), also known as CLN2, is a lysosomal serine protease detected in all tissues examined, with highest levels shown in heart and placenta. It is an aminopeptidase that cleaves tripeptides from the free N-terminus of polypeptides and may also have endopeptidase activity. Defects in TPP1 are the cause of classical late-infantile neuronal ceroid lipofusionosis, also known as ceroid lipofusionosis neuronal 2 (CLN2), a fatal childhood neurodegenerative disease characterized by progressive visual and mental decline, motor disturbance, epilepsy, and behavioral changes. |
运输条件 | Blue Ice |
存放说明 | 4℃ |
参考文献 |
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