추천 제품
생물학적 소스
mouse
결합
unconjugated
항체 형태
ascites fluid
항체 생산 유형
primary antibodies
클론
2F1, monoclonal
분자량
39 kDa
종 반응성
monkey, human
기술
direct ELISA: 1:10,000
flow cytometry: 1:200-1:400
immunohistochemistry: 1:200-1:1,000
western blot: 1:500-1:2,000
동형
IgG1
NCBI 수납 번호
배송 상태
wet ice
저장 온도
−20°C
타겟 번역 후 변형
unmodified
유전자 정보
human ... SMN1(6606)
일반 설명
Survival motor neuron 1 (SMN1), also known as gemin 1, is a survival motor neuron protein, which is highly expressed in spinal cord. SMN1 is localized on the telomeric region of human chromosome 5q13. The 38kDA protein is expressed mostly in nucleus and cytoplasm.
면역원
Purified recombinant fragment of human SMN1 expressed in E.coli.
Mouse monoclonal antibody raised against SMN1
Mouse monoclonal antibody raised against SMN1
생화학적/생리학적 작용
Survival motor neuron 1 (SMN1) is involved in the synthesis of full length survival motor neuron (SMN) mRNA transcripts. SMN1 also contributes to the biogenesis and assembly of snRNP (small nuclear riboproteins) in the cytoplasm, which plays an important role in pre-mRNA splicing or processing. Axonal SMN protein mediates the growth of axon and mRNA axonal transport. Mutations in the SMN1 gene has been associated with a genetic neurodegenerative disease called spinal muscular atrophy(SMA).
물리적 형태
Ascitic fluid containing 0.03% sodium azide.
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
10 - Combustible liquids
WGK
WGK 3
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.
SMN regulation in SMA and in response to stress: new paradigms and therapeutic possibilities
Human Genetics, 136(9), 1173-1191 (2017)
Identification and characterization of a spinal muscular atrophy-determining gene
Cell, 80(1), 155-165 (1995)
How do SMA-linked mutations of SMN1 lead to structural/functional deficiency of the SMA protein?
PLoS ONE, 12(6), e0178519-e0178519 (2017)
Different stability and proteasome-mediated degradation rate of SMN protein isoforms
PLoS ONE, 10(7), e0134163-e0134163 (2015)
SMN affects membrane remodelling and anchoring of the protein synthesis machinery
Journal of Cell Science, jcs-176750 (2016)
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