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Rabbit Anti-Patched/BF350 Conjugated antibody (bs-1614R-BF350)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-1614R-BF350
英文名称 Rabbit Anti-Patched/BF350 Conjugated antibody
中文名称 BF350标记的Patched/PTCH抗体
别    名 Protein patched homolog 1; PTCH; PTC1; A230106A15Rik; BCNS; FLJ26746; FLJ42602; Holoprosencephaly 7; HPE7; mes; NBCCS; OTTHUMP00000021709; OTTHUMP00000021710; Patched; Patched (Drosophila) homolog; Patched 1; Patched homolog (Drosophila); Patched homolog 1 (Drosophila); Patched homolog 1; Patched protein homolog 1; PTC; PTC1; PTCH; PTCH protein +12b; PTCH protein +4'; PTCH protein -10; PTCH protein; PTCH1; PTCH1 protein; PTCH11; Ptch2; ; Patched / PTCH; PTC1_HUMAN.   
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  发育生物学  神经生物学  信号转导  干细胞  转录调节因子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse,  (predicted: Rat, Chicken, Cow, Horse, Rabbit, )
产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 161kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Patched/PTCH
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
PTCH (Patched protein homolog 1) is a receptor for sonic hedgehog (SHH), indian hedgehog (IHH) and desert hedgehog (DHH). PTCH associates with the smoothened protein (SMO) to transduce the hedgehog's proteins signal.PTCH has a tumor suppressor function, as inactivation of this protein is probably a necessary, if not sufficient step for tumorigenesis. PTCH is expressed in the adult brain, lung, liver, heart, placenta, skeletal muscle, pancreas and kidney. It is also expressed in tumor cells but not in normal skin. During development PTCH is found in all major target tissues of sonic hedgehog, such as the ventral neural tube, somites, and tissues surrounding the zone of polarizing activity of the limb bud.
Defects in PTCH are probably the cause of basal cell nevus syndrome also known as Gorlin syndrome or Gorlin-Goltz syndrome.


Function:
Acts as a receptor for sonic hedgehog (SHH), indian hedgehog (IHH) and desert hedgehog (DHH). Associates with the smoothened protein (SMO) to transduce the hedgehog's proteins signal. Seems to have a tumor suppressor function, as inactivation of this protein is probably a necessary, if not sufficient step for tumorigenesis.

Subunit:
Interacts with SNX17. Interacts with IHH.

Subcellular Location:
Membrane.

Tissue Specificity:
In the adult, expressed in brain, lung, liver, heart, placenta, skeletal muscle, pancreas and kidney. Expressed in tumor cells but not in normal skin.

Post-translational modifications:
Glycosylation is necessary for SHH binding.

DISEASE:
Defects in PTCH1 are probably the cause of basal cell nevus syndrome (BCNS) [MIM:109400]; also known as Gorlin syndrome or Gorlin-Goltz syndrome. BCNS is an autosomal dominant disease characterized by nevoid basal cell carcinomas (NBCCS) and developmental abnormalities such as rib and craniofacial alterations, polydactyly, syndactyly, and spina bifida. In addition, the patients suffer from a multitude of tumors like basal cell carcinomas (BCC), fibromas of the ovaries and heart, cysts of the skin, jaws and mesentery, as well as medulloblastomas and meningiomas. PTCH1 is also mutated in squamous cell carcinoma (SCC). Could also be associated with large body size observed in BCNS patients.
Defects in PTCH1 are a cause of sporadic basal cell carcinoma (BCC) [MIM:605462].
Defects in PTCH1 are the cause of holoprosencephaly type 7 (HPE7) [MIM:610828]. Holoprosencephaly (HPE) [MIM:236100] is the most common structural anomaly of the brain, in which the developing forebrain fails to correctly separate into right and left hemispheres. Holoprosencephaly is genetically heterogeneous and associated with several distinct facies and phenotypic variability.

Similarity:
Belongs to the patched family.
Contains 1 SSD (sterol-sensing) domain.

Database links:

Entrez Gene: 5727 Human

Entrez Gene: 19206 Mouse

Entrez Gene: 89830 Rat

Omim: 601309 Human

SwissProt: Q13635 Human

SwissProt: Q86XG7 Human

SwissProt: Q61115 Mouse

Unigene: 494538 Human

Unigene: 228798 Mouse

Unigene: 102312 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

Ptch蛋白是细胞表面接受Hh信号蛋白的受体,目前主要用于肿瘤方面的研究。
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