| Long name: |
Myotilin Primary Polyclonal Antibody |
| Also known as: |
Myotilin PAb |
| Category: |
Primary Antibodies |
| Conjugation: |
Unconjugated |
| Target Antigen: |
Myotilin |
| Specificity: |
This is a highly specific antibody against Myotilin |
| Modification(s): |
None |
| Modification site(s): |
Unmodified antibody |
| Clonality: |
Polyclonal antibody |
| Clone number: |
Polyclonal antibody |
| Concentration: |
1ug per 1ul |
| Source: |
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Myotilin |
| Gene ID number: |
9499 |
| Tested Applications: |
IF(IHC-P), IHC-P, WB |
| Recommended dilutions: |
IF(IHC-P)(1:50-200), IHC-P(1:100-500), WB(1:100-1000) |
| Cross reactivity: |
Mouse, Rat, Human |
| Cross reactive species details: |
not every possible cross-reactivity is known, Due to limited amount of testing and knowledge |
| Background of the target antigen: |
Defects in the myotilin gene are reported to cause a form of autosomal dominant limb-girdle muscular dystrophy (LGMD), Myotilin is expressed in skeletal and cardiac muscle, Symptoms of adult onset LGMD are progressive weakness of the hip and shoulder girdles as well as a distinctive dysarthric pattern of speech, The muscle of affected individuals with LGMD shows degeneration of myofibers, a sarcomeric protein that is encoded by the gene mapping to human chromosome 5q31, and it co-localizes with a-actinin in the sarcomeric I-bands where it directly interacts with a-actinin, binds to a-actinin and is localized in the Z-line of myofibrils, centrally located myonuclei and an enhanced number of autophagic vesicles, fiber splitting, variations in fiber size, Myotilin |
| Purification method: |
This antibody was purified via Protein A |
| Storage conditions: |
50% glycerol and 0, Store at -20°, 09% sodium azide, C for up to 1 year, Keep the antibody in aqueous buffered solution containing 1% BSA |
| Synonym names: |
LGMD 1, LGMD1, MYOT, MYOTI_HUMAN, Myofibrillar titin like Ig domains protein, Myofibrillar titin-like Ig domains protein, Myotilin, TTID, TTID protein, Titin immunoglobulin domain protein, 57 kDa cytoskeletal protein |
| Properties: |
C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24° |
| Gene target: |
Myotilin |
| Short name: |
Myotilin Antibody |
| Technique: |
antibodies against human proteins, antibodies for, Antibody |
| Alternative name: |
Myotilin (Antibody to) |
| Alternative technique: |
antibodies |
| Identity: |
6573 |
| Gene: |
LGMD1 |
More about : LGMD1 |
| Long gene name: |
myotilin |
| Synonyms gene: |
TTID LGMD1A |
| Synonyms gene name: |
titin immunoglobulin domain protein (myotilin) limb-girdle muscular dystrophy 1A (autosomal dominant) |
| Synonyms: |
MYOT |
| Locus: |
5q31, 2 |
| Discovery year: |
1999-04-16 |
| GenBank acession: |
AF133820 |
| Entrez gene record: |
9499 |
| Pubmed identfication: |
10486214 10369880 |
| RefSeq identity: |
NM_006790 |
| Classification: |
I-set domain containing |
| Havana BLAST/BLAT: |
OTTHUMG00000129154 |
| Locus Specific Databases: |
Myotilin (TTID) sequence variations Leiden Muscular Dystrophy pages LRG_201 |