추천 제품
생물학적 소스
mouse
Quality Level
항체 형태
purified immunoglobulin
항체 생산 유형
primary antibodies
클론
4A8, monoclonal
종 반응성
human
기술
immunocytochemistry: suitable
western blot: suitable
동형
IgG1κ
NCBI 수납 번호
UniProt 수납 번호
배송 상태
wet ice
타겟 번역 후 변형
unmodified
유전자 정보
human ... MBNL1(4154)
일반 설명
Muscleblind 1 (MBNL1) is a member of the muscleblind family. MBNL1 is involved in pre-mRNA alternative splicing and acts as either splicing activator or repressor on specific pre-mRNA targets. This protein contains four C3H1-type zinc fingers and binds to expanded CUG repeat RNA. High expression of MBNL1 has been observed in cardiac and skeletal muscle as well as in differentiating myoblasts. MBNL1 is known to be involved in the pathogenesis of dystrophia myotonica type 1 (DM1). For those afflicted with this muscular disorder, MBNL1 is sequestered by DMPK RNAs that contain CUG triplet repeat expansions.
면역원
Full length recombinant protein corresponding to human MBNL1.
애플리케이션
Anti-MBNL1 Antibody, clone 4A8 is a Mouse Monoclonal Antibody for detection of MBNL1 also known as muscleblind-like protein 1, Triplet-expansion RNA-binding protein & has been validated in WB, ICC.
Immunocytochemistry Analysis:
1:4 dilution from a representative lot detected MBNL1 in myotonic dystrophy cells. Data courtesy of Dr. Ian Holt, Wolfson Centre for Inherited Neuromuscular Disease.
1:4 dilution from a representative lot detected MBNL1 in myotonic dystrophy cells. Data courtesy of Dr. Ian Holt, Wolfson Centre for Inherited Neuromuscular Disease.
품질
Evaluated by Western Blot in HEK293 cell lysate.
Western Blot Analysis: 1:10,000 dilution of this antibody detected MBNL1 on 10 µg of HEK293 cell lysate.
Western Blot Analysis: 1:10,000 dilution of this antibody detected MBNL1 on 10 µg of HEK293 cell lysate.
표적 설명
~39 kDa observed
물리적 형태
Format: Purified
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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.
Raphael I Benhamou et al.
Cell chemical biology, 27(2), 223-231 (2020-01-26)
Myotonic dystrophy type 2 (DM2) is a genetically defined disease caused by a toxic expanded repeat of r(CCUG) [r(CCUG)exp], harbored in intron 1 of CCHC-type zinc-finger nucleic acid binding protein (CNBP) pre-mRNA. This r(CCUG)exp causes toxicity via a gain-of-function mechanism
Raphael I Benhamou et al.
Journal of medicinal chemistry, 63(14), 7827-7839 (2020-07-14)
RNA repeat expansions are responsible for more than 30 incurable diseases. Among them is myotonic dystrophy type 1 (DM1), the most common form of adult on-set muscular dystrophy. DM1 is caused by an r(CUG) repeat expansion [r(CUG)exp] located in the
Fan Zhang et al.
Human molecular genetics, 26(16), 3056-3068 (2017-05-24)
Myotonic dystrophy Type 1 (DM1) is a rare genetic disease caused by the expansion of CTG trinucleotide repeats ((CTG)exp) in the 3' untranslated region of the DMPK gene. The repeat transcripts sequester the RNA binding protein Muscleblind-like protein 1 (MBNL1)
Alicia J Angelbello et al.
Cell chemical biology, 28(1), 34-45 (2020-11-07)
Many diseases are caused by toxic RNA repeats. Herein, we designed a lead small molecule that binds the structure of the r(CUG) repeat expansion [r(CUG)exp] that causes myotonic dystrophy type 1 (DM1) and Fuchs endothelial corneal dystrophy (FECD) and rescues
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