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Patched Rabbit pAb (bs-24459R)  
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產(chǎn)品編號 bs-24459R
英文名稱 Patched Rabbit pAb
中文名稱 Patched/PTCH抗體
別    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.  
研究領(lǐng)域 腫瘤  細(xì)胞生物  發(fā)育生物學(xué)  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  干細(xì)胞  轉(zhuǎn)錄調(diào)節(jié)因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,Rat (predicted: Human,Pig)
產(chǎn)品應(yīng)用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 161 kDa
檢測分子量
細(xì)胞定位 細(xì)胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Patched: 1-100/1447 <Cytoplasmic>
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
Q86XG7

Gene ID:
5727

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



Ptch蛋白是細(xì)胞表面接受Hh信號蛋白的受體,目前主要用于腫瘤方面的研究。
產(chǎn)品圖片
Sample: Lane 1: Cerebrum (Mouse) Lysate at 40 ug Lane 2: Cerebrum (Rat) Lysate at 40 ug Primary: Anti-Patched (bs-24459R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 161 kD Observed band size: 161 kD
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