同源盒蛋白SIX1抗體 返回列表頁

同源盒蛋白SIX1抗體

英文名稱 SIX1
中文名稱 同源盒蛋白SIX1抗體
別    名 BOS3; DFNA23; Homeobox protein SIX1; OTTHUMP00000179042; Sine oculis homeobox homolog 1; SIX homeobox 1; SIX1; SIX1_HUMAN; TIP39.

說 明 書 0.2ml  
研究領(lǐng)域 細(xì)胞生物  發(fā)育生物學(xué)  轉(zhuǎn)錄調(diào)節(jié)因子  表觀遺傳學(xué)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Cow, Rabbit, Sheep, 
產(chǎn)品應(yīng)用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 32kDa
細(xì)胞定位 細(xì)胞核 
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human SIX1
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.

PubMed PubMed
產(chǎn)品介紹 background:
The protein encoded by this gene is a homeobox protein that is similar to the Drosophila 'sine oculis' gene product. This gene is found in a cluster of related genes on chromosome 14 and is thought to be involved in limb development. Defects in this gene are a cause of autosomal dominant deafness type 23 (DFNA23) and branchiootic syndrome type 3 (BOS3). [provided by RefSeq, Jul 2008]

Function:
May be involved in limb tendon and ligament development.

Subcellular Location:
Nucleus.

Tissue Specificity:
Specifically expressed in skeletal muscle.

DISEASE:
Defects in SIX1 are the cause of deafness autosomal dominant type 23 (DFNA23) [MIM:605192]. A form of non-syndromic deafness characterized by prelingual, bilateral, symmetric hearing loss with a conductive component present in some but not all patients.
Defects in SIX1 are the cause of branchiootic syndrome type 3 (BOS3) [MIM:608389]. BOS3 is a syndrome characterized by usually bilateral branchial cleft fistulas or cysts, sensorineural and/or conductive hearing loss, pre-auricular pits, and structural defects of the outer, middle or inner ear. Otic defects include malformed and hypoplastic pinnae, a narrowed external ear canal, bulbous internal auditory canal, stapes fixation, malformed and hypoplastic cochlea. Branchial and otic anomalies are as those seen in individuals with the branchiootorenal syndrome. However, renal anomalies are absent in branchiootic syndrome patients.
Note=Defects in SIX1 could be a cause of branchiootorenal syndrome (BOR). BOR is an autosomal dominant disorder manifested by various combinations of preauricular pits, branchial fistulae or cysts, lacrimal duct stenosis, hearing loss, structural defects of the outer, middle, or inner ear, and renal dysplasia. Associated defects include asthenic habitus, long narrow facies, constricted palate, deep overbite, and myopia. Hearing loss may be due to mondini type cochlear defect and stapes fixation. Penetrance of BOR syndrome is high, although expressivity can be extremely variable.xy-11241R    AP2M1接頭相關(guān)蛋白復(fù)合體AP-2μ鏈1抗體
xy-11242R    Phospho-AP2M1 (Thr156)磷酸化接頭相關(guān)蛋白復(fù)合體AP-2μ鏈1抗體
xy-4901R    Annexin A7膜粘連蛋白7抗體
xy-9823R    C3orf153號染色體開放閱讀框15抗體
xy-6336R    APOB48R載脂蛋白B受體抗體
xy-6332R    ABCA2三磷酸腺苷結(jié)合盒轉(zhuǎn)運(yùn)蛋白2抗體
xy-6333R    ApoB 載脂蛋白B抗體
xy-6335R    ALOX15B花生四烯酸15脂氧合酶2抗體
xy-6338R    APOC4載脂蛋白C4抗體
xy-6337R    ARH低密度脂蛋白受體銜接蛋白抗體
xy-7550R    AUP1原始廣泛存在蛋白1抗體
xy-9398R    ANKRD37錨蛋白重復(fù)結(jié)構(gòu)域蛋白37抗體
xy-7588R    A4GALTα1,4-半乳糖基轉(zhuǎn)移酶1
xy-7642R    AGTRAP血管緊張素Ⅱ受體相關(guān)蛋白抗體
xy-7669R    AX2R膜粘連蛋白2受體抗體
xy-0203R    ACEI血管緊張素1轉(zhuǎn)換酶抑制劑抗體
xy-1229R    AATF拮抗凋亡轉(zhuǎn)錄因子抗體
xy-0094R    AACT-Alpha1α-1抗胰糜蛋白酶抗體
xy-0096R    alpha 1 Antitrypsinα-1抗胰蛋白酶抗體
xy-1224R    ABCB6ATP結(jié)合蛋白家族6抗體
xy-1231R    ABCG1三磷酸腺苷結(jié)合盒亞家族G1抗體
xy-0611R    Adiponectin receptor 2脂聯(lián)素受體2抗體
xy-9741R    ANGEL1ANGEL1蛋白抗體
xy-9742R    ANGEL2ANGEL2蛋白抗體
xy-9743R    ANKLE2ANKLE2蛋白抗體
xy-9744R    ANKMY1睪丸特異性錨樣蛋白1抗體
xy-9745R    ANKRD13B錨蛋白重復(fù)結(jié)構(gòu)域蛋白13B抗體
xy-9746R    ANKRD20A1錨蛋白重復(fù)結(jié)構(gòu)域蛋白20A1抗體
xy-9747R    ANKRD20A3錨蛋白重復(fù)結(jié)構(gòu)域蛋白20A3抗體
xy-9748R    ANKRD22錨蛋白重復(fù)結(jié)構(gòu)域蛋白22抗體
xy-9751R    ANKRD50錨蛋白重復(fù)結(jié)構(gòu)域蛋白50抗體
xy-9753R    ATP6V1G2氫離子轉(zhuǎn)運(yùn)ATP合成酶6G抗體
xy-9755R    ANKRD26錨蛋白重復(fù)結(jié)構(gòu)域蛋白26抗體