FITC标记的免疫球蛋白μ链结合蛋白2抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的免疫球蛋白μ链结合蛋白2抗体

FITC标记的免疫球蛋白μ链结合蛋白2抗体

商家询价

产品名称： FITC标记的免疫球蛋白μ链结合蛋白2抗体

英文名称： Anti-SMUBP2/FITC

产品编号： HZ-11757R-FITC

产品价格： null

产品产地： 中国/上海

品牌商标： HZbscience

更新时间： 2023-08-17T10:24:20

使用范围： ICC=1:50-200 IF=1:50-200

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 Rabbit Anti-SMUBP2/FITC Conjugated antibody

FITC标记的免疫球蛋白μ链结合蛋白2抗体

 

产品编号 bs-11757R-FITC
英文名称 Anti-SMUBP2/FITC
中文名称 FITC标记的免疫球蛋白μ链结合蛋白2抗体
别    名 AEP; Antifreeze enhancer binding protein; ATP-dependent helicase IGHMBP2; Cardiac transcription factor 1; Cardiac transcription factor1; CATF 1; CATF1; DNA-binding protein SMUBP-2; GF-1; Glial factor 1; HCSA; HMN 6; HMN6; IGHMBP 2; Ighmbp2; Immunoglobulin mu binding protein 2; Immunoglobulin mu binding protein2; Immunoglobulin mu-binding protein 2; Immunoglobulin S mu binding protein 2; Immunoglobulin S mu binding protein2; RIPE3 b1; RIPE3b 1; RIPE3b1; SMARD 1; SMARD1; SMBP2_HUMAN; SMUBP 2.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 心血管  细胞生物  神经生物学  信号转导  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Pig, Cow, 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 109kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SMUBP2 (271-355aa)
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:
IGHMBP2 is a 993 amino acid nuclear and cytoplasmic protein that is ubiquitously expressed. Belonging to the DNA2/NAM7 helicase family, IGHMBP2 is a 5' to 3' helicase that unwinds RNA and DNA duplexes in an ATP-dependent reaction. IGHMBP2 also acts as a transcriptional regulator and is necessary for transcriptional activation of the flounder liver-type antifreeze protein gene. IGHMBP2 exists as a homooligomer and is part of the cytosolic ribonucleoprotein complex. Mutations in the gene encoding IGHMBP2 are suggested to lead to distal hereditary motor neuronopathy type 6 (HMN6), also known as spinal muscular atrophy distal autosomal recessive 1 (DSMA1) or spinal muscular atrophy with respiratory distress 1 (SMARD1). HMN6 is characterized by weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs and severe respiratory distress.

Function:
5' to 3' helicase that unwinds RNA and DNA duplices in an ATP-dependent reaction. Acts as a transcription regulator. Required for the transcriptional activation of the flounder liver-type antifreeze protein gene. Exhibits strong binding specificity to the enhancer element B of the flounder antifreeze protein gene intron. Binds to the insulin II gene RIPE3B enhancer region. May be involved in translation (By similarity). DNA-binding protein specific to 5'-phosphorylated single-stranded guanine-rich sequence related to the immunoglobulin mu chain switch region. Preferentially binds to the 5'-GGGCT-3' motif. Interacts with tRNA-Tyr. Stimulates the transcription of the human neurotropic virus JCV.

Subunit:
Homooligomer. Interacts with RUVBL1, RUVBL2, GTF3C1 andABT1. Is part of large cytosolic ribonucleoprotein complexes(Probable). Associates with the ribosomes.

Subcellular Location:
Nucleus. Cytoplasm.

Tissue Specificity:
Expressed in all tissues examined.

Post-translational modifications:
Phosphorylated upon DNA damage, probably by ATM or ATR.

DISEASE:
Defects in IGHMBP2 are the cause of distal hereditary motor neuronopathy type 6 (HMN6) [MIM:604320]; also known as spinal muscular atrophy distal autosomal recessive 1 (DSMA1) or spinal muscular atrophy with respiratory distress 1 (SMARD1). Distal hereditary motor neuronopathies constitute a heterogeneous group of neuromuscular disorders caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs. The most prominent symptoms of HMN6 are severe respiratory distress resulting from diaphragmatic paralysis with eventration shown on chest x-ray and predominant involvement of the upper limbs and distal muscles.

Similarity:
Belongs to the DNA2/NAM7 helicase family.
Contains 1 AN1-type zinc finger.
Contains 1 R3H domain.

Database links:
UniProtKB/Swiss-Prot: P38935.3

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

IGHMPB2是一个993个氨基酸的核和胞质蛋白，广泛表达。属于Dn2/NAM7解旋酶家族，IGHMPP2是5’至3’的解旋酶，在ATP依赖性反应中释放RNA和DNA双链。IGHMbp2也作为转录调控因子，是牙鲆肝脏型抗冻蛋白基因转录激活所必需的。IGHMbP2作为同质寡聚体存在，是胞浆核糖核蛋白复合物的一部分。编码IGHMP2基因的突变提示远端型遗传性运动神经病变6型（HMN6），也称为脊髓性肌萎缩，远端常染色体隐性遗传1（DSMA1）或脊髓性肌萎缩症伴呼吸窘迫1（SMARD1）。HMN6的特点是腿部胫前和腓侧肌的远端肌肉无力和严重的呼吸窘迫。