FHL1 sirna
FHL1 siRNA (Mouse)
NCBI and Uniprot Product Information
Uniprot Description
FHL1: May have an involvement in muscle development or hypertrophy. Defects in FHL1 are the cause of X-linked dominant scapuloperoneal myopathy (SPM). 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). 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). 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). This disorder is allelic to severe early-onset reducing body myopathy (RBM). 5 isoforms of the human protein are produced by alternative splicing.
Protein type: Cell development/differentiation
Cellular Component: focal adhesion; cytoplasm; plasma membrane; nucleus
Molecular Function: zinc ion binding; metal ion binding
Biological Process: multicellular organismal development; positive regulation of potassium ion transport; negative regulation of cell growth; cell differentiation
Research Articles on FHL1
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Product Notes
The FHL1 fhl1 (Catalog #AAA8224687) is a siRNA produced from Synthetic and is intended for research purposes only. The product is available for immediate purchase. The FHL1 siRNA (Mouse) reacts with Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's FHL1 can be used in a range of immunoassay formats including, but not limited to, RNA Interference (RNAi). Researchers should empirically determine the suitability of the FHL1 fhl1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "FHL1, siRNA" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
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