Key features and details
- Goat polyclonal to FHL1
- Suitable for: ELISA, WB, ICC/IF, IHC-Fr
- Reacts with: Mouse, Human, Pig
- Isotype: IgG
Product nameAnti-FHL1 antibody
See all FHL1 primary antibodies
DescriptionGoat polyclonal to FHL1
Specificityab26072 is specific for SLIMMER / FHL1 isoform B.
Tested applicationsSuitable for: ELISA, WB, ICC/IF, IHC-Frmore details
Species reactivityReacts with: Mouse, Human, Pig
- Human Heart lysate
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage bufferpH: 7.30
Preservative: 0.02% Sodium azide
Constituents: Tris buffered saline, 0.05% BSA
Concentration information loading...
PurityImmunogen affinity purified
Purification notesPurified by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Our Abpromise guarantee covers the use of ab26072 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 0.01 - 0.1 µg/ml. Predicted molecular weight: 36 kDa.
A 1 hour primary incubation is recommended for this product.
|ICC/IF||Use at an assay dependent concentration.|
|IHC-Fr||Use at an assay dependent concentration.|
FunctionMay have an involvement in muscle development or hypertrophy.
Tissue specificityIsoform 1 is highly expressed in skeletal muscle and to a lesser extent in heart, placenta, ovary, prostate, testis, small intestine, colon and spleen. Expression is barely detectable in brain, lung, liver, kidney, pancreas, thymus and peripheral blood leukocytes. Isoform 2 is expressed in brain, skeletal muscle and to a lesser extent in heart, colon, prostate and small intestine. Isoform 3 is expressed in testis, heart and skeletal muscle.
Involvement in diseaseDefects in FHL1 are the cause of X-linked dominant scapuloperoneal myopathy (SPM) [MIM:300695]. Scapuloperoneal syndrome (SPS) was initially described more than 120 years ago by Jules Broussard as 'une forme hereditaire d'atrophie musculaire progressive' beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm. The etiology of this condition remains unclear.
Defects in FHL1 are the cause of X-linked myopathy with postural muscle atrophy (XMPMA) [MIM:300696]. Myopathies are inherited muscle disorders characterized by weakness and atrophy of voluntary skeletal muscle, and several types of myopathy also show involvement of cardiac muscle. XMPMA is a distinct form of adult-onset X-linked recessive myopathy with several features in common with other myopathies, but the presentation of a pseudoathletic phenotype, scapuloperoneal weakness, and bent spine is unique and might render the clinical phenotype distinguishable from other myopathies.
Defects in FHL1 are the cause of X-linked severe early-onset reducing body myopathy (RBM) [MIM:300717]. RBM is a rare muscle disorder causing progressive muscular weakness and characteristic intracytoplasmic inclusions in myofibers. Clinical presentations of RBM have ranged from early onset fatal to childhood onset to adult onset cases.
Defects in FHL1 are the cause of X-linked childhood-onset reducing body myopathy (CO-RBM) [MIM:300718]. This disorder is allelic to severe early-onset reducing body myopathy (RBM) [MIM:300717].
Sequence similaritiesContains 3 LIM zinc-binding domains.
Developmental stageElevated levels during postnatal muscle growth.
Cellular localizationCytoplasm; Cytoplasm. Nucleus and Nucleus. Cytoplasm > cytosol. Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.
- Information by UniProt
- bA535K18.1 antibody
- FHL 1 antibody
- FHL 1B antibody
ab26072 has been referenced in 1 publication.
- Cottle DL et al. SLIMMER (FHL1B/KyoT3) interacts with the proapoptotic protein Siva-1 (CD27BP) and delays skeletal myoblast apoptosis. J Biol Chem 284:26964-77 (2009). WB, ICC/IF, IHC-Fr ; Mouse . PubMed: 19643733