| Long name: |
ALEXA FLUOR 594 Conjugated, Myotilin Polyclonal Antibody |
| Also known as: |
Anti-Myotilin PAb ALEXA FLUOR 594 |
| Category: |
Conjugated Primary Antibodies |
| Conjugated with: |
594, ALEXA FLUOR® |
| Host Organism: |
Rabbit (Oryctolagus cuniculus) |
| Target Antigen: |
Myotilin |
| Specificity: |
This is a highly specific antibody against Myotilin |
| Modification: |
Unmodified |
| Modification Site: |
None |
| Clonality: |
Polyclonal |
| Clone: |
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) |
| Recommended dilutions: |
IF(IHC-P)(1:50-200) |
| Crossreactivity: |
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 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: |
Purified by Protein A |
| Storage conditions: |
50% glycerol and 0, Keep refrigerated at 2 to 8 degrees Celcius for up to one year, 09% sodium azide, Store this antibody in aqueous buffered solution containing 1% BSA |
| Excitation emission: |
590nm/617nm |
| Synonyms: |
MYOT |
| Properties: |
ALEXA FLUOR they should be stored frozen at - 24°, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies, C, C for long term storage and for short term at + 5°, For facs or microscopy Alexa 1 conjugate |
| Conjugation: |
594, ALEXA FLUOR®, Alexa Fluor |
| Conjugated: |
Alexa conjugate 1 |
| Gene target: |
Myotilin |
| Short name: |
Myotilin Antibody |
| Technique: |
antibodies against human proteins, antibodies for, Antibody |
| Label: |
ALEXA FLUOR 594 |
| Alternative name: |
ALEXA FLUOR 594, 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) |
| 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 |