Key features and details
- Rabbit polyclonal to FHL1
- Suitable for: WB, IHC-P, ICC/IF
- Reacts with: Mouse, Human
- Isotype: IgG
Product nameAnti-FHL1 antibody
See all FHL1 primary antibodies
DescriptionRabbit polyclonal to FHL1
Tested applicationsSuitable for: WB, IHC-P, ICC/IFmore details
Species reactivityReacts with: Mouse, Human
Predicted to work with: Rat, Sheep, Rabbit, Horse, Guinea pig, Cow, Dog
- WB: Fetal muscle lysate. IHC-P: Human kidney epithelial cells.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.09% Sodium azide
Constituents: 2% Sucrose, PBS
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab49241 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 0.1 µg/ml. Detects a band of approximately 30 kDa (predicted molecular weight: 32 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.|
|IHC-P||Use a concentration of 4 - 8 µg/ml.|
|ICC/IF||Use a concentration of 1 µg/ml.|
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
Anti-FHL1 antibody (ab49241) at 0.1 µg/ml + Human Fetal muscle lysate at 10 µg
HRP conjugated anti-Rabbit IgG at 1/50000 dilution
Predicted band size: 32 kDa
Observed band size: 30 kDa why is the actual band size different from the predicted?
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human kidney tissue labelling FHL1 with ab49241 at 4-8µg/ml. Arrows indicate positively labelled epithelial renal tubule cells. Magnification: 400X.
ICC/IF image of ab49241 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab49241, 1µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
ab49241 has been referenced in 5 publications.
- Malfatti E et al. Skeletal muscle biopsy analysis in reducing body myopathy and other FHL1-related disorders. J Neuropathol Exp Neurol 72:833-45 (2013). PubMed: 23965743
- Matulis CK & Mayo KE The LIM domain protein FHL2 interacts with the NR5A family of nuclear receptors and CREB to activate the inhibin-a subunit gene in ovarian granulosa cells. Mol Endocrinol 26:1278-90 (2012). Human . PubMed: 22734036
- Feldkirchner S et al. Patient-specific protein aggregates in myofibrillar myopathies: Laser microdissection and differential proteomics for identification of plaque components. Proteomics : (2012). PubMed: 23044792
- Veith C et al. Paxillin regulates pulmonary arterial smooth muscle cell function in pulmonary hypertension. Am J Pathol 181:1621-33 (2012). WB . PubMed: 22959909
- Selcen D et al. Reducing bodies and myofibrillar myopathy features in FHL1 muscular dystrophy. Neurology 77:1951-9 (2011). PubMed: 22094483