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Rabbit Anti-Hairless/PE Conjugated antibody (bs-8507R-PE)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-8507R-PE
英文名称 Rabbit Anti-Hairless/PE Conjugated antibody
中文名称 PE标记的无毛发蛋白抗体
别    名 HR; ALUNC; AU; HAIR_HUMAN; Hairless protein; Host range; HSA277165; Protein hairless.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 染色质和核信号  转录调节因子  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 127kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Hairless
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:
Hairless is a 1,189 amino acid protein which is expressed as two isoforms produced by alternative splicing. The two isoforms are expressed in a variety of tissues in varying concentrations. Isoform 1 is more abundant than isoform 2 and is expressed at low levels in kidneys and testis, while isoform 2 is expressed abundantly in skin. Both isoforms are also present together in many tissues and are expressed strongly in small intestine and brain and weakly in trachea. HR is thought to be a transcription factor involved in hair growth. Hair growth occurs in three phases known as anagen, catagen and telogen, which are phases where growth, regression and rest, respectively, are taking place. By unknown mechanisms, HR is thought to regulate one of the hair growth phases and to work with vitamin D receptor (VDR) to regulate hair follicle cycling. Defects in HR may cause two serious ailments, known as alopecia universalis congenita (ALUNC) and atrichia with papular lesions (APL), which is also referred to as congenital atrichia. Both are autosomally recessive impairments. ALUNC is a rare condition in which hair follicles are produced without hair, while APL is a serious disease in which papillary lesions may cover the body and little to no hair is grown.

Function:
May act as a transcription factor that could act on to regulate one of the phases of hair growth.

Subcellular Location:
Nucleus.

Tissue Specificity:
Strongest expression of isoforms 1 and 2 is seen in the small intestine, weaker expression in brain and colon, and trace expression is found in liver, pancreas, spleen, thymus, stomach, salivary gland, appendix and trachea. Isoform 1 is always the most abundant. Isoform 1 is exclusively expressed at low levels in kidney and testis. Isoform 2 is exclusively expressed at high levels in the skin.

DISEASE:
Defects in HR are the cause of alopecia universalis congenita (ALUNC) [MIM:203655]. ALUNC is a rare autosomal recessive form of hair loss characterized by hair follicles without hair.
Defects in HR are the cause of atrichia with popular lesions (APL) [MIM:209500]; also known as congenital atrichia. APL is an autosomal recessive disease characterized by papillary lesions over most of the body and almost complete absence of hair.
Defects in HR are the cause of hypotrichosis type 4 (HYPT4) [MIM:146550]. An autosomal dominant condition characterized by reduced amount of hair, alopecia, little or no eyebrows, eyelashes or body hair, and coarse, wiry, twisted hair in early childhood.

Similarity:
Contains 1 JmjC domain.

Database links:
 

UniProtKB/Swiss-Prot: O43593.5



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