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Rabbit Anti-Tyrosine Hydroxylase/BF594 Conjugated antibody (bs-0016R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-0016R-BF594
英文名稱 Rabbit Anti-Tyrosine Hydroxylase/BF594 Conjugated antibody
中文名稱 BF594標記的酪氨酸羥化酶抗體
別    名 Tyrosine Hydroxylase; DYT14; DYT5b; ple; Protein Pale; c; The; TYH; Tyrosine 3 hydroxylase; Tyrosine 3 monooxygenase; Tyk2; TY3H_HUMAN; Tyrosine 3-monooxygenase; Tyrosine 3-hydroxylase; TH.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  免疫學  神經生物學  信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse,  (predicted: Rat, Dog, Cow, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 60kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TH
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 background:
The protein encoded by this gene is involved in the conversion of tyrosine to dopamine. It is the rate-limiting enzyme in the synthesis of catecholamines, hence plays a key role in the physiology of adrenergic neurons. Mutations in this gene have been associated with autosomal recessive Segawa syndrome. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008]

Function:
Plays an important role in the physiology of adrenergic neurons.

Tissue Specificity:
Mainly expressed in the brain and adrenal glands.

DISEASE:
Defects in TH are the cause of Segawa syndrome autosomal recessive (ARSEGS) [MIM:605407]. A form of DOPA-responsive dystonia presenting in infancy or early childhood. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. Some cases present with parkinsonian symptoms in infancy. Unlike all other forms of dystonia, it is an eminently treatable condition, due to a favorable response to L-DOPA.
Note=May play a role in the pathogenesis of Parkinson disease (PD). A genome-wide copy number variation analysis has identified a 34 kilobase deletion over the TH gene in a PD patient but not in any controls.

Similarity:
Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.

Database links:

Entrez Gene: 7054 Human

Entrez Gene: 21823 Mouse

Entrez Gene: 25085 Rat

Omim: 191290 Human

SwissProt: P07101 Human

SwissProt: P24529 Mouse

SwissProt: P04177 Rat

Unigene: 435609 Human

Unigene: 1292 Mouse

Unigene: 11082 Rat



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

神經細胞標志物
酪氨酸羥化酶(TH)是兒茶酚胺類神經遞質即多巴胺、去甲腎上腺素、腎上腺素生物合成過程所需的限速酶,它以四氫生物喋呤啶(BH4)為輔酶,催化酪氨酸的羥化而生成多巴(DOPA)。
已知在患帕金森病(Parkinson disease,PD)時,腦內多巴胺(dopamine,DA)的減少與此酶活性低下有關。因此對PD模型動物來說,若將TH基因植入腦內,便可以提高腦內DA水平而達到基因治療目的。
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