Search Thermo Fisher Scientific
Search Thermo Fisher Scientific
应用 | 建议稀释比 | 已发表文章 |
---|---|---|
免疫印迹 (WB) |
1:32,000 | - |
免疫细胞化学 (ICC/IF) |
Assay-dependent | - |
免疫沉淀 (IP) |
Assay-dependent | - |
产品规格 | |
---|---|
种属反应 |
Human |
宿主/亚型 |
Mouse / IgG1 |
分类 |
Monoclonal |
类型 |
Antibody |
克隆号 |
5H8-B4 |
抗原 |
Human Lamin A Ig-fold |
偶联物 |
Unconjugated |
形式 |
Liquid |
浓度 |
1 mg/mL |
纯化类型 |
Protein G |
保存液 |
PBS |
内含物 |
0.1% sodium azide |
保存条件 |
Maintain refrigerated at 2-8°C for up to 1 month. For long term storage store at -20°C |
RRID |
AB_2539446 |
This antibody recognizes the FPLD-associated lamin A/C mutant strain R482W.
Lamins are a class of intermediate filament proteins that form a matrix on the inner surface of the nuclear envelope. These proteins are found in many different cell types in three different forms (A, B, and C). Lamins A and C are alternatively spliced versions of the LMNA gene. The LMNA gene has been linked to many disorders of the muscular system, nervous system, and the fat distributions systems including: Emery-Dreifuss muscular dystrophy, Dunnigan-type familial partial lipodystrophy (FPLD), limb-girdle muscular dystrophy (LGMD1B), dilated cardiomyopathy (CMD1A), axonal neuropathy (Charcot-Marie-Tooth disease; CMT2B1), and mandibuloacral dysplasia (MAD).
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: 70 kDa lamin; Lamin A+C mutant; lamin a-c; lamin A/C-like 1; Lamin AC; Lamin-A/C; mutant 453W; Mutant lamin A/C; Prelamin-A/C; progerin mutant; Renal carcinoma antigen NY-REN-32; RP11-54H19.1
基因别名: CDCD1; CDDC; CMD1A; CMT2B1; EMD2; FPL; FPLD; FPLD2; HGPS; IDC; LDP1; LFP; LGMD1B; LMN1; LMNA; LMNC; LMNL1; PRO1
UniProt ID: (Human) P02545
Entrez Gene ID: (Human) 4000