추천 제품
생물학적 소스
mouse
Quality Level
항체 형태
purified immunoglobulin
항체 생산 유형
primary antibodies
클론
2B1, monoclonal
종 반응성
human
종 반응성(상동성에 의해 예측)
Xenopus, mouse
기술
immunocytochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable
동형
IgG1κ
NCBI 수납 번호
UniProt 수납 번호
배송 상태
wet ice
타겟 번역 후 변형
unmodified
유전자 정보
human ... SMA4(11039)
일반 설명
The survival of motor neurons (SMN) protein is essential for the biogenesis of small nuclear RNA (snRNA)-ribonucleoproteins (snRNPs), the major components of the pre-mRNA splicing machinery. Though it is ubiquitously expressed, SMN deficiency causes the motor neuron degenerative disease spinal muscular atrophy (SMA). SMN deficiency has unexpected cell type-specific effects on the repertoire of snRNAs and mRNAs. It alters the stoichiometry of snRNAs and causes widespread pre-mRNA splicing defects in numerous transcripts of diverse genes, preferentially those containing a large number of introns, in SMN-deficient mouse tissues. The SMN complex plays a role in RNA metabolism and in splicing regulation.
특이성
This antibody recognizes SMN.
면역원
Epitope: Unknown
His-tagged recombinant protein corresponding to human SMN.
애플리케이션
Anti-SMN Antibody, clone 2B1 is a Mouse Monoclonal Antibody for detection of SMN also known as Spinal muscular atrophy (Werdnig-Hoffmann disease Kugelberg-Welander disease) & has been validated in WB, ICC & IP.
Immunocytochemistry Analysis: 1:500 dilution from a representative lot detected SMN in HeLa cells.
Research Category
Epigenetics & Nuclear Function
Epigenetics & Nuclear Function
Research Sub Category
RNA Metabolism & Binding Proteins
RNA Metabolism & Binding Proteins
RNA Metabolism & Binding Proteins
RNA Metabolism & Binding Proteins
품질
Evaluated by Western Blot in HeLa cell lysate.
Western Blot Analysis: 0.5 µg/ml of this antibody detected SMN on 10 µg of HeLa cell lysate.
Western Blot Analysis: 0.5 µg/ml of this antibody detected SMN on 10 µg of HeLa cell lysate.
표적 설명
35 kDa was observed; however, the calculated molecular weight is 31.849 kDa
물리적 형태
Format: Purified
Protein G Purified
Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.05% sodium azide.
저장 및 안정성
Stable for 1 year at 2-8°C from date of receipt.
분석 메모
Control
HeLa cell lysate
HeLa cell lysate
기타 정보
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
면책조항
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Storage Class Code
12 - Non Combustible Liquids
WGK
WGK 1
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
시험 성적서(COA)
제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.
Minoru Satoh et al.
Arthritis and rheumatism, 63(7), 1972-1978 (2011-03-23)
Autoantibodies in the systemic rheumatic diseases are clinically useful biomarkers of the diagnosis or of certain clinical characteristics. An unusual pattern of immunoprecipitation, in which the D, E, F, and G proteins of small nuclear RNPs (snRNP) but without other
Elena Hernandez-Gerez et al.
Annals of clinical and translational neurology, 7(9), 1580-1593 (2020-08-14)
The purpose of the study was to determine the extent and role of systemic hypoxia in the pathogenesis of spinal muscular atrophy (SMA). Hypoxia was assayed in vivo in early-symptomatic (postnatal day 5) SMA-model mice by pimonidazole and [18 F]-Fluoroazomycin
Lea Marie Esser et al.
International journal of molecular sciences, 24(21) (2023-11-14)
The survival motor neuron (SMN) complex is a multi-megadalton complex involved in post-transcriptional gene expression in eukaryotes via promotion of the biogenesis of uridine-rich small nuclear ribonucleoproteins (UsnRNPs). The functional center of the complex is formed from the SMN/Gemin2 subunit.
Lu Chen et al.
Nucleic acids research, 50(21), 12400-12424 (2022-08-11)
Trimethylguanosine synthase 1 (TGS1) is a highly conserved enzyme that converts the 5'-monomethylguanosine cap of small nuclear RNAs (snRNAs) to a trimethylguanosine cap. Here, we show that loss of TGS1 in Caenorhabditis elegans, Drosophila melanogaster and Danio rerio results in
Phosphorylation of pICln by the autophagy activating kinase ULK1 regulates snRNP biogenesis and splice activity of the cell.
Esser, et al.
Computational and structural biotechnology journal, 21, 2100-2109 (2023)
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