Rabbit anti-Human FHL Polyclonal Antibody | anti-FHL1 antibody

Anti-FHL Antibody

Cat. Num. AAA1752976

• Gene Names: FHL1; KYOT; SLIM; FCMSU; FHL-1; FHL1A; FHL1B; FLH1A; SLIM1; XMPMA; RBMX1A; RBMX1B; SLIM-1; SLIMMER
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry, Immunocytochemistry, Immunofluorescence, Flow Cytometry, Functional Assay, ELISA
• Purity: Immunogen affinity purified.
• Synonyms: FHL; Polyclonal Antibody; Anti-FHL Antibody; Four and a half LIM domains protein 1; FHL-1; Skeletal muscle LIM-protein 1; SLIM; SLIM-1; FHL1; SLIM1; anti-FHL1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: No cross reactivity with other proteins.
• Purity/Purification: Immunogen affinity purified.
• Form/Format: Lyophilized; Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
• Sequence Length: 296

• Applicable Applications for anti-FHL1 antibody: Western Blot (WB), Immunohistochemistry (IHC) Paraffin, Immunocytochemistry (ICC), Immunofluorescence (IF), Flow Cytometry (FC/FACS), Direct ELISA (EIA)
• Application Notes: WB: 0.25-0.5ug/ml (Human); IHC-P (Embedded Section): 0.5-1ug/ml (Human); ICC: 2ug/ml (Human); IF: 2ug/ml (Human); FC/FACS: 1-3ug/1x106 cells (Human); Direct ELISA: 0.1-0.5ug/ml (Human); Tested Species: In-house tested species with positive results.; By Heat: Boiling the paraffin sections in 10mM citrate buffer, pH6.0, for 20mins is required for the staining of formalin/paraffin sections.
• Immunogen: E Coli-derived human FHL1 recombinant protein (Position: A2-P323).
• Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
• Preparation and Storage: Store at -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen store at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Flow Cytometry (FC/FACS)

Immunofluorescence (IF)

Immunohistochemistry (IHC)

Immunohistochemistry (IHC)

Western Blot (WB)

Related Product Information for anti-FHL1 antibody

Description: Rabbit IgG polyclonal antibody for FHL detection. Tested with WB, IHC-P, ICC/IF, FCM, Direct ELISA in Human.

Background: Four and a half LIM domains protein 1 is a protein that in humans is encoded by the FHL1 gene. It is mapped to Xq26.3. This gene encodes a member of the four-and-a-half-LIM-only protein family. Family members contain two highly conserved, tandemly arranged, zinc finger domains with four highly conserved cysteines binding a zinc atom in each zinc finger. Expression of these family members occurs in a cell- and tissue-specific mode and these proteins are involved in many cellular processes. Mutations in this gene have been found in patients with Emery-Dreifuss muscular dystrophy. Multiple alternately spliced transcript variants which encode different protein isoforms have been described.

References

1. Morgan MJ, Madgwick AJ (Aug 1996). "Slim defines a novel family of LIM-proteins expressed in skeletal muscle". Biochemical and Biophysical Research Communications. 225 (2): 632-8.
2. Lee SM, Tsui SK, Chan KK, Garcia-Barcelo M, Waye MM, Fung KP, Liew CC, Lee CY (Aug 1998). "Chromosomal mapping, tissue distribution and cDNA sequence of four-and-a-half LIM domain protein 1 (FHL1)". Gene. 216 (1): 163-70.
3. Windpassinger C, Schoser B, Straub V, Hochmeister S, Noor A, Lohberger B, Farra N, Petek E, Schwarzbraun T, Ofner L, Löscher WN, Wagner K, Lochmüller H, Vincent JB, Quasthoff S (Jan 2008). "An X-linked myopathy with postural muscle atrophy and generalized hypertrophy, termed XMPMA, is caused by mutations in FHL1". American Journal of Human Genetics. 82 (1): 88-99. doi:10.1016/j.ajhg.2007.09.004.

NCBI and Uniprot Product Information

• NCBI GI #: 228480205
• NCBI GeneID: 2273
• NCBI Accession #: NP_001153171.1
• NCBI GenBank Nucleotide #: NP_001153171.1
• UniProt Accession #: Q13642
• NCBI Official Full Name: four and a half LIM domains protein 1 isoform 5
• NCBI Official Synonym Full Names: four and a half LIM domains 1
• NCBI Official Symbol: FHL1
• NCBI Official Synonym Symbols: KYOT; SLIM; FCMSU; FHL-1; FHL1A; FHL1B; FLH1A; SLIM1; XMPMA; RBMX1A; RBMX1B; SLIM-1; SLIMMER
• NCBI Protein Information: four and a half LIM domains protein 1
• UniProt Protein Name: Four and a half LIM domains protein 1
• Protein Family: Fork head transcription factor
• UniProt Gene Name: FHL1
• UniProt Synonym Gene Names: SLIM1; FHL-1; SLIM; SLIM-1
• UniProt Entry Name: FHL1_HUMAN

NCBI Description

This gene encodes a member of the four-and-a-half-LIM-only protein family. Family members contain two highly conserved, tandemly arranged, zinc finger domains with four highly conserved cysteines binding a zinc atom in each zinc finger. Expression of these family members occurs in a cell- and tissue-specific mode and these proteins are involved in many cellular processes. Mutations in this gene have been found in patients with Emery-Dreifuss muscular dystrophy. Multiple alternately spliced transcript variants which encode different protein isoforms have been described.[provided by RefSeq, Nov 2009]

Uniprot Description

FHL1 iso1: 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

Chromosomal Location of Human Ortholog: Xq26

Cellular Component: focal adhesion; cytoplasm; plasma membrane; nucleus; cytosol

Molecular Function: protein binding; zinc ion binding

Biological Process: muscle development; organ morphogenesis; positive regulation of potassium ion transport; negative regulation of cell growth; cell differentiation

Disease: Myopathy, Reducing Body, X-linked, Early-onset, Severe; Myopathy, X-linked, With Postural Muscle Atrophy; Myopathy, Reducing Body, X-linked, Childhood-onset; Scapuloperoneal Myopathy, X-linked Dominant