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Rabbit Anti-Sox3/PE-Cy7 Conjugated antibody (bs-10794R-PE-Cy7)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-10794R-PE-Cy7
英文名称 Rabbit Anti-Sox3/PE-Cy7 Conjugated antibody
中文名称 PE-Cy7标记的转录因子Sox3抗体
别    名 Sox-3; GHDX; Infundibular hypoplasia and hypopituitarism; MRGH; PHP; SOXB; SRY Box 3; SRY related HMG box gene 3; SRY Sex Determining Region Y Box 3; Transcription factor SOX-3; SOX3_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  信号转导  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 Human, Mouse,  (predicted: Rat, Chicken, Sheep, )
产品应用 ICC=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 45kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Sox3
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:
Sox genes comprise a family of genes that are related to the mammalian sex determining gene SRY. These genes similarly contain sequences that encode for the HMG-box domain, which is responsible for the sequence-specific DNA-binding activity. Sox genes encode putative transcriptional regulators implicated in the decision of cell fates during development and the control of diverse developmental processes. The highly complex group of Sox genes cluster at least 40 different loci that rapidly diverged in various animal lineages. At present, 30 Sox genes have been identified. Members of this family have been shown to be conserved during evolution and to play key roles during animal development. Some are involved in human diseases, including sex reversal. Sox-3, also known as MRGH or SOXB, is implicated in mental retardation X-linked with isolated growth hormone deficiency (MRXGH) and infundibular hypoplasia and hypopituitarism.

Function:
SOX 3 is also known as SRY related HMG BOX gene 3. All SOX proteins have a single HMG box. Humans with mutations in SOX3 have panhypopituitarism. Conditional disruption of SOX3 in mice demonstrates that anterior pituitary development depends on SOX3 expression in the overlying neural ectoderm.

Subunit:
Interacts with SOX2 and FGFR1

Subcellular Location:
Nuclear.

DISEASE:
Defects in SOX3 are a cause of panhypopituitarism X-linked (PHPX) [MIM:312000]. Affected individuals have absent infundibulum, anterior pituitary hypoplasia, and ectopic posterior pituitary.
Defects in SOX3 are the cause of mental retardation X-linked with isolated growth hormone deficiency (MRXGH) [MIM:300123].
Defects in SOX3 are the cause of 46,XX sex reversal type 3 (SRXX3) [MIM:300833]. A condition in which male gonads develop in a genetic female (female to male sex reversal). Note=Copy number variations (CNV) encompassing or in close proximity to SOX3 are responsible for XX male reversal. These variations include two duplications of approximately 123 kb and 85 kb, the former of which spans the entire SOX3 gene; a 343 kb deletion immediately upstream of SOX3 that is probably responsible of altered regulation (and not increased dosage) of SOX3; a large (approximately 6 Mb) duplication that encompasses SOX3 and at least 18 additional distally located genes. Its proximal breakpoint falls within the SOX3 regulatory region. This large rearrangement has been found in a patient with XX male reversal and a complex phenotype that also includes a scrotal hypoplasia, microcephaly, developmental delay, and growth retardation.

Similarity:
Contains 1 HMG box DNA-binding domain.

Database links:

Entrez Gene: 6658 Human

Entrez Gene: 101030174 Monkey

Entrez Gene: 20675 Mouse

Omim: 313430 Human

SwissProt: P41225 Human

SwissProt: P53784 Mouse

Unigene: 157429 Human

Unigene: 35784 Mouse



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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