NF1 Rabbit pAb (一抗) | Bioss
货号：bs-4140R
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概述
产品编号
bs-4140R
产品类型
宠物抗体、农牧业/家禽抗体
英文名称
NF1 Rabbit pAb
中文名称
1型神经纤维瘤抗体
英文别名
NFNS; VRNF; WSS; Dsk9; E030030H24Rik; Mhdadsk9; Nf-1; NF1_HUMAN; NF1; Neurofibromatosis-related protein NF-1; NF1_MOUSE; NF1_RAT; neurofibromin 1; neurofibromatosis; von Recklinghausen disease; Watson disease
抗体来源
Rabbit
免疫原
KLH conjugated synthetic peptide derived from human Neurofibromin 1: 2701-2839/2839
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Polyclonal
理论分子量
147/319 kDa
浓度
1mg/ml
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
研究领域
Cancer > Cancer Metabolism > Response to hypoxia
Epigenetics and Nuclear Signaling > Transcription > Cancer susceptibility > Tumor Suppressors
Metabolism > Pathways and Processes > Metabolism processes > Hypoxia
Neuroscience > Neurotransmission > Intracellular Signaling > Cytoskeletal
Signal Transduction > Signaling Pathway > G Protein Signaling > Small G Proteins
SWISS
Gene ID
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
数据库链接
产品介绍
神经纤维素蛋白首先发现于神经细胞，是一种肿瘤抑制蛋白，通过调控Ras基因控制异常细胞生长，并且在cAMP信号传导通路中起调节作用.
神经纤维瘤Ⅰ型(neurofibromatosis type 1,NF1)是一种由内分泌紊乱引起的神经纤维瘤,属于常染色体显性遗传病，其发病率为1/3500，主要表现为咖啡斑、神经纤维瘤、Lisch结节(虹膜错构瘤)等。每3，500个新生儿中就有一个是神经纤维细胞瘤I型患者，其临床表现为表皮或皮下多发性神经纤维瘤，良性多于恶性，常沿神经干分布。有时，神经纤维瘤会长大，或者发展到脑和脊髓，大约有一半以上患者智力低下。
神经纤维瘤Ⅰ型(neurofibromatosis type 1,NF1)是一种由内分泌紊乱引起的神经纤维瘤,属于常染色体显性遗传病，其发病率为1/3500，主要表现为咖啡斑、神经纤维瘤、Lisch结节(虹膜错构瘤)等。每3，500个新生儿中就有一个是神经纤维细胞瘤I型患者，其临床表现为表皮或皮下多发性神经纤维瘤，良性多于恶性，常沿神经干分布。有时，神经纤维瘤会长大，或者发展到脑和脊髓，大约有一半以上患者智力低下。
背景资料
Neurofibromin is a product of the tumor suppressor gene, Neurofibromatosis type I. Neurofibromin is known to have GTPase activity that modulates the ras pathway. The absence of or alteration of the neurofibromin protein may lead to Neurofibromatosis disease. This protein has not been purified, therefore, most of the information regarding this protein has been deduced from homology analysis of its gene sequence.
产品应用
| 应用 | 已检合格种属 | 预测种属 | 推荐稀释比例 |
|---|---|---|---|
| IHC-P | Human, Mouse | Rat, Rabbit, Dog, Horse | 1:100-500 |
| IHC-F | Human, Mouse | Rat, Rabbit, Dog, Horse | 1:100-500 |
| IF | Human, Mouse | Rat, Rabbit, Dog, Horse | 1:100-500 |
| Flow-Cyt | Mouse | Human, Rat, Rabbit, Dog, Horse | 1μg/Test |
| ICC/IF | Human | Mouse, Rat, Rabbit, Dog, Horse | 1:100-500 |
交叉反应
交叉反应: Human, Mouse (predicted: Rat, Rabbit, Dog, Horse)
相关产品
暂无相关产品
靶标
基因名
NF1
蛋白名
Neurofibromin
疾病
Neurofibromatosis 1 (NF1) [MIM:162200]: A disease characterized by patches of skin pigmentation (cafe-au-lait spots), Lisch nodules of the iris, tumors in the peripheral nervous system and fibromatous skin tumors. Individuals with the disorder have increased susceptibility to the development of benign and malignant tumors. Note=The disease is caused by mutations affecting the gene represented in this entry.
Leukemia, juvenile myelomonocytic (JMML) [MIM:607785]: An aggressive pediatric myelodysplastic syndrome/myeloproliferative disorder characterized by malignant transformation in the hematopoietic stem cell compartment with proliferation of differentiated progeny. Patients have splenomegaly, enlarged lymph nodes, rashes, and hemorrhages. Note=The disease is caused by mutations affecting the gene represented in this entry.
Watson syndrome (WS) [MIM:193520]: A syndrome characterized by the presence of pulmonary stenosis, cafe-au-lait spots, and mental retardation. It is considered as an atypical form of neurofibromatosis. Note=The disease is caused by mutations affecting the gene represented in this entry.
Familial spinal neurofibromatosis (FSNF) [MIM:162210]: Considered to be an alternative form of neurofibromatosis, showing multiple spinal tumors. Note=The disease is caused by mutations affecting the gene represented in this entry.
Neurofibromatosis-Noonan syndrome (NFNS) [MIM:601321]: Characterized by manifestations of both NF1 and Noonan syndrome (NS). NS is a disorder characterized by dysmorphic facial features, short stature, hypertelorism, cardiac anomalies, deafness, motor delay, and a bleeding diathesis. Note=The disease is caused by mutations affecting the gene represented in this entry.
Colorectal cancer (CRC) [MIM:114500]: A complex disease characterized by malignant lesions arising from the inner wall of the large intestine (the colon) and the rectum. Genetic alterations are often associated with progression from premalignant lesion (adenoma) to invasive adenocarcinoma. Risk factors for cancer of the colon and rectum include colon polyps, long-standing ulcerative colitis, and genetic family history. Note=The gene represented in this entry may be involved in disease pathogenesis.
Leukemia, juvenile myelomonocytic (JMML) [MIM:607785]: An aggressive pediatric myelodysplastic syndrome/myeloproliferative disorder characterized by malignant transformation in the hematopoietic stem cell compartment with proliferation of differentiated progeny. Patients have splenomegaly, enlarged lymph nodes, rashes, and hemorrhages. Note=The disease is caused by mutations affecting the gene represented in this entry.
Watson syndrome (WS) [MIM:193520]: A syndrome characterized by the presence of pulmonary stenosis, cafe-au-lait spots, and mental retardation. It is considered as an atypical form of neurofibromatosis. Note=The disease is caused by mutations affecting the gene represented in this entry.
Familial spinal neurofibromatosis (FSNF) [MIM:162210]: Considered to be an alternative form of neurofibromatosis, showing multiple spinal tumors. Note=The disease is caused by mutations affecting the gene represented in this entry.
Neurofibromatosis-Noonan syndrome (NFNS) [MIM:601321]: Characterized by manifestations of both NF1 and Noonan syndrome (NS). NS is a disorder characterized by dysmorphic facial features, short stature, hypertelorism, cardiac anomalies, deafness, motor delay, and a bleeding diathesis. Note=The disease is caused by mutations affecting the gene represented in this entry.
Colorectal cancer (CRC) [MIM:114500]: A complex disease characterized by malignant lesions arising from the inner wall of the large intestine (the colon) and the rectum. Genetic alterations are often associated with progression from premalignant lesion (adenoma) to invasive adenocarcinoma. Risk factors for cancer of the colon and rectum include colon polyps, long-standing ulcerative colitis, and genetic family history. Note=The gene represented in this entry may be involved in disease pathogenesis.
相似性
Contains 1 CRAL-TRIO domain.
Contains 1 Ras-GAP domain.
Contains 1 Ras-GAP domain.
功能
Stimulates the GTPase activity of Ras. NF1 shows greater affinity for Ras GAP, but lower specific activity. May be a regulator of Ras activity.
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