产品订购信息：
英文名称  Anti-SMN1 

中文名称   运动神经元生存蛋白1说明书 

别    名  Component of gems 1; Component of gems 2; Gemin 1; Gemin-1; SMA; SMA1; SMA3; SMN; SMN_HUMAN; SMN1; SMN2; SMNC; SMNT; Survival motor neuron protein; survival of motor neuron 1, telomeric; survival of motor neuron 2, centromeric.

浓    度   1mg/1ml

规 格  0.1ml/100μg 0.2ml/200μg

抗体来源   Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Dog 

产品类型   一抗  

研究领域    细胞生物 免疫学 染色质和核信号 表观遗传学

蛋白分子量  predicted molecular weight: 32kDa

性    状   Lyophilized or Liquid

免 疫 原 KLH conjugated synthetic peptide derived from human SMN1 

亚    型   IgG

纯化方法   affinity purified by Protein A

储 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

运动神经元生存蛋白1说明书 产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500

（石蜡切片需做抗原修复） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

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

产品介绍 SMN1 is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7, which is thought to be an exon splice enhancer. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The full length protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Four transcript variants encoding distinct isoforms have been described.

Function : The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing in the nucleus. It may also play a role in the metabolism of snoRNPs.

Subunit : Component of an import snRNP complex composed of KPNB1, RNUT1, SMN1 and ZNF259. Part of the core SMN complex that contains SMN1, GEMIN2/SIP1, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Interacts with DDX20, FBL, NOLA1, RNUT1, SYNCRIP and with several spliceosomal snRNP core Sm proteins, including SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE and ILF3. Interacts with OSTF1, LSM10 and LSM11.

Subcellular Location : Cytoplasm. Nucleus, gem. Note=Localized in subnuclear structures next to coiled bodies, called Gemini of Cajal bodies (Gems).

Tissue Specificity : Expressed in a wide variety of tissues. Expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. Also seen at high levels in spinal cord. Present in osteoclasts and mononuclear cells (at protein level).

DISEASE : Spinal muscular atrophy 1 (SMA1) [MIM:253300]: A form of spinal muscular atrophy, a group of neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Autosomal recessive forms are classified according to the age of onset, the maximum muscular activity achieved, and survivorship. The severity of the disease is mainly determined by the copy number of SMN2, a copy gene which predominantly produces exon 7-skipped transcripts and only low amount of full-length transcripts that encode for a protein identical to SMN1. Only about 4% of SMA patients bear one SMN1 copy with an intragenic mutation. SMA1 is a severe form, with onset before 6 months of age. SMA1 patients never achieve the ability to sit. Note=The disease is caused by mutations affecting the gene represented in this entry.

Spinal muscular atrophy 2 (SMA2) [MIM:253550]: An autosomal recessive form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. It has intermediate severity, with onset between 6 and 18 months. Patients do not reach the motor milestone of standing, and survive into adulthood. Note=The disease is caused by mutations affecting the gene represented in this entry.

Spinal muscular atrophy 3 (SMA3) [MIM:253400]: An autosomal recessive form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Onset is after 18 months. Patients develop ability to stand and walk and survive into adulthood. Note=The disease is caused by mutations affecting the gene represented in this entry.

Spinal muscular atrophy 4 (SMA4) [MIM:271150]: An autosomal recessive form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Onset is in adulthood, disease progression is slow, and patients can stand and walk. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity : Belongs to the SMN family.

Contains 1 Tudor domain.

Database links : UniProtKB/Swiss-Prot: Q16637.1

P19ARF p19ARF基因抗原Multi-class antibodies规格： 0.5mg

Anti-ARAlpha1/ADRA1B alpha 1能受体抗体Multi-class antibodies规格： 0.1ml

Rhesus antibody Rh phospho-arfaptin 2(Ser260) 磷酸化ADP核糖基化因子结合蛋白2抗体 规格 0.1ml

TIMP-1 浓缩液 0.1ml 进口分装

Uridine Phosphorylase 1 英文名称： 尿嘧啶核苷磷酸化酶1抗体 0.2ml

DGCR2 英文名称： DGCR2蛋白抗体 0.2ml

Anti-ARAlpha1/ADRA1B alpha 1能受体抗体Multi-class antibodies规格： 0.1ml

Anti-PDGF-B/FITC 荧光素标记血小板源性生长因子-B抗体IgGMulti-class antibodies规格： 0.2ml

Rabbit Anti- guinea pig IgG/FITC 荧光素标记兔抗豚鼠IgGMulti-class antibodies规格： 0.3ml

TNF-α膜受体抗体/DR3 死亡受体3抗体 Anti-DR3/TNF-αR/TRAMP 0.1ml

Rabbit Anti-horse IgG/PE-Cy3 PE-Cy3标记的兔抗马IgG 0.1ml

FBXO11 英文名称： F-box蛋白家族FBXO11抗体 0.2ml

Rhesus antibody Rh Phospho-Torc2/Crtc2(Ser171) 磷酸化CREB转录共激活因子TORC2抗体 规格 0.1ml

Rabbit Anti- guinea pig IgG/FITC 荧光素标记兔抗豚鼠IgGMulti-class antibodies规格： 0.3ml

phospho-SYN1(Ser603) 英文名称： 磷酸化神经突触素1抗体 0.1ml

phospho-Ephrin B (Ty*9) 英文名称： 磷酸化Ephrin B抗体 0.1ml

新型基因抗体 Anti-TBX2 0.1ml

Anti-TLK1/FITC 荧光素标记调节染色体组装激酶1抗体IgGMulti-class antibodies规格： 0.2ml

Rhesus antibody Rh phospho-LKB1(Ser428) 磷酸化丝氨酸/苏氨酸蛋白激酶抗体 规格 0.1ml

NR2B (Glutamate receptor) 谷氨酸受体(抗原)Multi-class antibodies规格： 0.5mg

6-10B, 人细胞系

MS4A1 Others Human 人 CD20 / MS4A1 (aa 213-297) 人细胞裂解液 (阳性对照)

大鼠表皮黑色素细胞完全培养基 100mL

LLC-MK2恒河猴肾细胞 Ganges RIver LLC-MK2 monkey kidney cells RPMI-1640(GIBCO)+10%FBS

IFNA4 Protein Human 重组人 IFNα4 / IFNa4 / Ierferon alpha-4 蛋白 (His 标签)

MDA-MB-175VII(人导管癌细胞) 5×106cells/瓶×2 BRL 3A(大鼠肝细胞)

6-10B, 人细胞系

MS4A1 Others Human 人 CD20 / MS4A1 (aa 213-297) 人细胞裂解液 (阳性对照)

大鼠表皮黑色素细胞完全培养基 100mL

LLC-MK2恒河猴肾细胞 Ganges RIver LLC-MK2 monkey kidney cells RPMI-1640(GIBCO)+10%FBS

IFNA4 Protein Human 重组人 IFNα4 / IFNa4 / Ierferon alpha-4 蛋白 (His 标签)

MDA-MB-175VII(人导管癌细胞) 5×106cells/瓶×2 BRL 3A(大鼠肝细胞)

运动神经元生存蛋白1说明书 FCAR Others Rat 大鼠 FCAR / CD89 人细胞裂解液 (阳性对照)

间充质干细胞培养基MSCM

GHS1 金黄地鼠皮肤成纤维细胞

CM-M022小鼠甲状腺上皮细胞完全培养基100mL

LX-2, 人肝星形细胞株

EB病毒转化的人B淋巴细胞;KMY0906 人内脏脂肪细胞完全培养基 100mL

抗体的生物素化标记实验要点：

1. 运动神经元生存蛋白1说明书 如在反应混合液中有叠氮钠或游离氨基存在,会抑制标记反应。因此,蛋白质在反应前要对 0.1mol/L碳酸氢钠缓冲液或0.5mol/L硼酸缓冲液充分透析；

2.所用的NHSB及待生物素化蛋白质之间的分子比按蛋白质表面的ε-氨基的密度会有所不同,选择不当则影响标记的效率,应先用几个不同的分子比来筛选最适条件；

3.用NHSB量过量也是不利的,抗原的结合位点可能因此被封闭,导致抗体失活；

4.由于抗体的氨基不易接近可能造成生物素化不足,此时可加入去污剂如 Triton x-100, Tween20等；

5.当游离ε-氨基(赖氨酸残基的氨基)存在于抗体的抗原结合位点时,或位于酶的催化位点时,生物素化会降低或损伤抗体蛋白的结合力或活性；

6.生物素还可能与不同的功能基团,如羰基、氨基、巯基、异咪唑基及苯酚基,也可与糖基共价结合；

7.交联反应后,应充分透析,否则,残余的生物素会对生物素化抗体与亲和素的结合产生竞争作用；

8.在细胞的荧光标记实验中,中和亲和素的本底低,但由于链霉亲和素含有少量正电荷,故对某些细胞可导致高本底。

抗体的鉴定：

1）运动神经元生存蛋白1说明书 抗体的效价鉴定：不管是用于诊断还是用于,制备抗体的目的都是要求较高效价。不同的抗原制备的抗体,要求的效价不一。鉴定效价的方法很多,包括有试管凝集反应,琼脂扩散试验,酶联免疫吸附试验等。常用的抗原所制备的抗体一般都有约成的鉴定效价的方法,以资比较。如制备抗抗体的效价,一般就采用琼脂扩散试验来鉴定。

2）抗体的特异性鉴定：抗体的特异性是指与相应抗原或近似抗原物质的识别能力。抗体的特异性高,它的识别能力就强。衡量特异性通常以交叉反应率来表示。交叉反应率可用竞争抑制试验测定。以不同浓度抗原和近似抗原分别做竞争抑制曲线,计算各自的结合率,求出各自在IC50时的浓度,并按公式计算交叉反应率。 

如果所用抗原浓度IC50浓度为pg/管,而一些近似抗原物质的IC50浓度几乎是无穷大时,表示这一抗血清与其他抗原物质的交叉反应率近似为0,即该血清的特异性较好。

3）抗体亲和力：是指抗体和抗原结合的牢固程度。亲和力的高低是由抗原分子的大小,抗体分子的结合位点与抗原决定簇之间立体构型的合适度决定的。有助于维持抗原抗体复合物稳定的分子间力有氢键,疏水键,侧链相反电荷基因的库仑力,范德华力和空间斥力。亲和力常以亲和常数K表示,K的单位是L/mol。抗体亲和力的测定对抗体的筛选,确定抗体的用途,验证抗体的均一性等均有重要意义。