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| 產(chǎn)地 | 進口、國產(chǎn) |
| 品牌 | 上海莼試 |
| 保存條件 | Store at -20 °C |
| 貨號 | CS11300 |
| 應用范圍 | 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 |
| CAS編號 | |
| 抗體名 | Anti-SMN1 |
| 克隆性 | |
| 靶點 | 詳見說明書 |
| 適應物種 | 詳見說明書 |
| 形態(tài) | 詳見說明書 |
| 宿主 | 詳見說明書 |
| 亞型 | IgG |
| 標識物 | 詳見說明書 |
| 濃度 | 1mg/1ml% |
| 免疫原 | KLH conjugated synthetic peptide derived from human SMN1 |
產(chǎn)品訂購信息：
英文名稱 Anti-SMN1
中文名稱 運動神經(jīng)元生存蛋白1說明書
別 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.
濃 度 1mg/1ml
規(guī) 格 0.1ml/100μg 0.2ml/200μg
抗體來源 Rabbit
克隆類型 polyclonal
交叉反應 Human, Mouse, Rat, Dog
產(chǎn)品類型 一抗
研究領域 細胞生物 免疫學 染色質(zhì)和核信號 表觀遺傳學
蛋白分子量 predicted molecular weight: 32kDa
性 狀 Lyophilized or Liquid
免 疫 原 KLH conjugated synthetic peptide derived from human SMN1
亞 型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide
運動神經(jīng)元生存蛋白1說明書 產(chǎn)品應用 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
（石蠟切片需做抗原修復）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產(chǎn)品介紹 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規(guī)格： 0.5mg
Anti-ARAlpha1/ADRA1B alpha 1能受體抗體Multi-class antibodies規(guī)格： 0.1ml
Rhesus antibody Rh phospho-arfaptin 2(Ser260) 磷酸化ADP核糖基化因子結(jié)合蛋白2抗體 規(guī)格 0.1ml
TIMP-1 濃縮液 0.1ml 進口分裝
Uridine Phosphorylase 1 英文名稱： 尿嘧啶核苷磷酸化酶1抗體 0.2ml
DGCR2 英文名稱： DGCR2蛋白抗體 0.2ml
Anti-ARAlpha1/ADRA1B alpha 1能受體抗體Multi-class antibodies規(guī)格： 0.1ml
Anti-PDGF-B/FITC 熒光素標記血小板源性生長因子-B抗體IgGMulti-class antibodies規(guī)格： 0.2ml
Rabbit Anti- guinea pig IgG/FITC 熒光素標記兔抗豚鼠IgGMulti-class antibodies規(guī)格： 0.3ml
TNF-α膜受體抗體/DR3 死亡受體3抗體 Anti-DR3/TNF-αR/TRAMP 0.1ml
Rabbit Anti-horse IgG/PE-Cy3 PE-Cy3標記的兔抗馬IgG 0.1ml
FBXO11 英文名稱： F-box蛋白家族FBXO11抗體 0.2ml
Rhesus antibody Rh Phospho-Torc2/Crtc2(Ser171) 磷酸化CREB轉(zhuǎn)錄共激活因子TORC2抗體 規(guī)格 0.1ml
Rabbit Anti- guinea pig IgG/FITC 熒光素標記兔抗豚鼠IgGMulti-class antibodies規(guī)格： 0.3ml
phospho-SYN1(Ser603) 英文名稱： 磷酸化神經(jīng)突觸素1抗體 0.1ml
phospho-Ephrin B (Ty*9) 英文名稱： 磷酸化Ephrin B抗體 0.1ml
新型基因抗體 Anti-TBX2 0.1ml
Anti-TLK1/FITC 熒光素標記調(diào)節(jié)染色體組裝激酶1抗體IgGMulti-class antibodies規(guī)格： 0.2ml
Rhesus antibody Rh phospho-LKB1(Ser428) 磷酸化絲氨酸/蘇氨酸蛋白激酶抗體 規(guī)格 0.1ml
NR2B (Glutamate receptor) 谷氨酸受體(抗原)Multi-class antibodies規(guī)格： 0.5mg
6-10B, 人細胞系
MS4A1 Others Human 人 CD20 / MS4A1 (aa 213-297) 人細胞裂解液 (陽性對照)
大鼠表皮黑色素細胞完全培養(yǎng)基 100mL
LLC-MK2恒河猴腎細胞 Ganges RIver LLC-MK2 monkey kidney cells RPMI-1640(GIBCO)+10%FBS
IFNA4 Protein Human 重組人 IFNα4 / IFNa4 / Ierferon alpha-4 蛋白 (His 標簽)
MDA-MB-175VII(人導管癌細胞) 5×106cells/瓶×2 BRL 3A(大鼠肝細胞)
6-10B, 人細胞系
MS4A1 Others Human 人 CD20 / MS4A1 (aa 213-297) 人細胞裂解液 (陽性對照)
大鼠表皮黑色素細胞完全培養(yǎng)基 100mL
LLC-MK2恒河猴腎細胞 Ganges RIver LLC-MK2 monkey kidney cells RPMI-1640(GIBCO)+10%FBS
IFNA4 Protein Human 重組人 IFNα4 / IFNa4 / Ierferon alpha-4 蛋白 (His 標簽)
MDA-MB-175VII(人導管癌細胞) 5×106cells/瓶×2 BRL 3A(大鼠肝細胞)
運動神經(jīng)元生存蛋白1說明書 FCAR Others Rat 大鼠 FCAR / CD89 人細胞裂解液 (陽性對照)
間充質(zhì)干細胞培養(yǎng)基MSCM
GHS1 金黃地鼠皮膚成纖維細胞
CM-M022小鼠甲狀腺上皮細胞完全培養(yǎng)基100mL
LX-2, 人肝星形細胞株
EB病毒轉(zhuǎn)化的人B淋巴細胞;KMY0906 人內(nèi)臟脂肪細胞完全培養(yǎng)基 100mL
抗體的生物素化標記實驗要點：
1. 運動神經(jīng)元生存蛋白1說明書 如在反應混合液中有疊氮鈉或游離氨基存在,會抑制標記反應。因此,蛋白質(zhì)在反應前要對 0.1mol/L碳酸氫鈉緩沖液或0.5mol/L硼酸緩沖液充分透析；
2.所用的NHSB及待生物素化蛋白質(zhì)之間的分子比按蛋白質(zhì)表面的ε-氨基的密度會有所不同,選擇不當則影響標記的效率,應先用幾個不同的分子比來篩選最適條件；
3.用NHSB量過量也是不利的,抗原的結(jié)合位點可能因此被封閉,導致抗體失活；
4.由于抗體的氨基不易接近可能造成生物素化不足,此時可加入去污劑如 Triton x-100, Tween20等；
5.當游離ε-氨基(賴氨酸殘基的氨基)存在于抗體的抗原結(jié)合位點時,或位于酶的催化位點時,生物素化會降低或損傷抗體蛋白的結(jié)合力或活性；
6.生物素還可能與不同的功能基團,如羰基、氨基、巰基、異咪唑基及苯酚基,也可與糖基共價結(jié)合；
7.交聯(lián)反應后,應充分透析,否則,殘余的生物素會對生物素化抗體與親和素的結(jié)合產(chǎn)生競爭作用；
8.在細胞的熒光標記實驗中,中和親和素的本底低,但由于鏈霉親和素含有少量正電荷,故對某些細胞可導致高本底。
抗體的鑒定：
1）運動神經(jīng)元生存蛋白1說明書 抗體的效價鑒定：不管是用于診斷還是用于,制備抗體的目的都是要求較高效價。不同的抗原制備的抗體,要求的效價不一。鑒定效價的方法很多,包括有試管凝集反應,瓊脂擴散試驗,酶聯(lián)免疫吸附試驗等。常用的抗原所制備的抗體一般都有約成的鑒定效價的方法,以資比較。如制備抗抗體的效價,一般就采用瓊脂擴散試驗來鑒定。
2）抗體的特異性鑒定：抗體的特異性是指與相應抗原或近似抗原物質(zhì)的識別能力?？贵w的特異性高,它的識別能力就強。衡量特異性通常以交叉反應率來表示。交叉反應率可用競爭抑制試驗測定。以不同濃度抗原和近似抗原分別做競爭抑制曲線,計算各自的結(jié)合率,求出各自在IC50時的濃度,并按公式計算交叉反應率。
如果所用抗原濃度IC50濃度為pg/管,而一些近似抗原物質(zhì)的IC50濃度幾乎是無窮大時,表示這一抗血清與其他抗原物質(zhì)的交叉反應率近似為0,即該血清的特異性較好。
3）抗體親和力：是指抗體和抗原結(jié)合的牢固程度。親和力的高低是由抗原分子的大小,抗體分子的結(jié)合位點與抗原決定簇之間立體構(gòu)型的合適度決定的。有助于維持抗原抗體復合物穩(wěn)定的分子間力有氫鍵,疏水鍵,側(cè)鏈相反電荷基因的庫侖力,范德華力和空間斥力。親和力常以親和常數(shù)K表示,K的單位是L/mol?？贵w親和力的測定對抗體的篩選,確定抗體的用途,驗證抗體的均一性等均有重要意義。
