Application

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.

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.

General description

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.

Immunogen

Full length recombinant protein corresponding to human MBNL1.

Physical form

Format: Purified

Quality

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.

Target description

~39 kDa observed