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Rabbit Anti-PAX3/PE-Cy5.5 Conjugated antibody (bs-1097R-PE-Cy5.5)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-1097R-PE-Cy5.5
英文名稱 Rabbit Anti-PAX3/PE-Cy5.5 Conjugated antibody
中文名稱 PE-Cy5.5標記的配對盒基因3抗體
別    名 CDHS; HUP 2; HUP2; MGC120381; MGC120382; MGC120383; MGC120384; MGC134778; paired box homeotic gene 3; Paired box protein Pax 3; Paired box protein Pax3; paired domain gene 3; paired domain gene HuP2; PAX 3; PAX3/FKHR fusion gene; Waardenburg syndrome 1; WS 1; WS1.   
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  發育生物學  信號轉導  干細胞  轉錄調節因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat, 
產品應用 Flow-Cyt=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 53kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PAX3
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:
This protein is a member of the paired box (PAX) family of transcription factors. Members of the PAX family typically contain a paired box domain and a paired-type homeodomain. These proteins play critical roles during fetal development. Mutations in paired box gene 3 are associated with Waardenburg syndrome, craniofacial-deafness-hand syndrome, and alveolar rhabdomyosarcoma. The translocation t(2;13)(q35;q14), which represents a fusion between PAX3 and the forkhead gene, is a frequent finding in alveolar rhabdomyosarcoma. Alternative splicing results in transcripts encoding isoforms with different C-termini.

Function:
Transcription factor that may regulate cell proliferation, migration and apoptosis. Involved in neural development and myogenesis.

Subunit:
Can bind to DNA as a homodimer or a heterodimer with PAX7. Interacts with PAXBP1; the interaction links PAX3 to a WDR5-containing histone methyltransferase complex. Interacts with DAXX.

Subcellular Location:
Nucleus.

DISEASE:
Waardenburg syndrome 1 (WS1) [MIM:193500]: WS1 is an autosomal dominant disorder characterized by non-progressive sensorineural deafness, pigmentary disturbances such as frontal white blaze of hair, heterochromia of irides, white eyelashes, leukoderma, and wide bridge of nose owing to lateral displacement of the inner canthus of each eye (dystopia canthorum). WS1 shows variable clinical expression and some affected individuals do not manifest hearing impairment or iris pigmentation disturbances. Dystopia canthorum is the most consistent sign and is found in 98% of the patients. Note=The disease is caused by mutations affecting the gene represented in this entry.
Waardenburg syndrome 3 (WS3) [MIM:148820]: WS3 is an autosomal dominant disorder characterized by sensorineural deafness, pigmentary disturbances, dystopia canthorum and limb anomalies such as hypoplasia of the musculoskeletal system, flexion contractures, fusion of the carpal bones, syndactylies. Note=The disease is caused by mutations affecting the gene represented in this entry.
Craniofacial-deafness-hand syndrome (CDHS) [MIM:122880]: Thought to be an autosomal dominant disease which comprises absence or hypoplasia of the nasal bones, hypoplastic maxilla, small and short nose with thin nares, limited movement of the wrist, short palpebral fissures, ulnar deviation of the fingers, hypertelorism and profound sensory-neural deafness. Note=The disease is caused by mutations affecting the gene represented in this entry.
Rhabdomyosarcoma 2 (RMS2) [MIM:268220]: A form of rhabdomyosarcoma, a highly malignant tumor of striated muscle derived from primitive mesenchymal cells and exhibiting differentiation along rhabdomyoblastic lines. Rhabdomyosarcoma is one of the most frequently occurring soft tissue sarcomas and the most common in children. It occurs in four forms: alveolar, pleomorphic, embryonal and botryoidal rhabdomyosarcomas. Note=The gene represented in this entry is involved in disease pathogenesis. A chromosomal aberration involving PAX3 is found in rhabdomyosarcoma. Translocation (2;13)(q35;q14) with FOXO1. The resulting protein is a transcriptional activator.
Note=A chromosomal aberration involving PAX3 is a cause of rhabdomyosarcoma. Translocation t(2;2)(q35;p23) with NCOA1 generates the NCOA1-PAX3 oncogene consisting of the N-terminus part of PAX3 and the C-terminus part of NCOA1. The fusion protein acts as a transcriptional activator. Rhabdomyosarcoma is the most common soft tissue carcinoma in childhood, representing 5-8% of all malignancies in children.

Similarity:
Belongs to the paired homeobox family.
Contains 1 homeobox DNA-binding domain.
Contains 1 paired domain.

Database links:

Entrez Gene: 5077 Human

Entrez Gene: 18505 Mouse

Entrez Gene: 114502 Rat

Omim: 606597 Human

SwissProt: P23760 Human

SwissProt: P24610 Mouse

SwissProt: Q0IH87 Xenopus laevis

SwissProt: Q645N4 Xenopus laevis

Unigene: 42146 Human

Unigene: 1371 Mouse

Unigene: 225252 Rat



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

同源結構域蛋白(Homeodomain Proteins)
PAX-3屬于轉錄抑制因子。在胚胎發育和腫瘤生長中起關鍵作用。其突變和某些腫瘤的發病有關。
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