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

FHL1, CT (Four And A Half LIM Domains Protein 1, FHL-1, Skeletal Muscle LIM-protein 1, SLIM 1, SLIM, SLIM1) (FITC)

Cat. Num. AAA6475959

• Gene Names: FHL1; KYOT; SLIM; FHL-1; FHL1A; FHL1B; FLH1A; SLIM1; XMPMA; SLIM-1; SLIMMER
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: FHL1; Polyclonal Antibody; CT (Four And A Half LIM Domains Protein 1; FHL-1; Skeletal Muscle LIM-protein 1; SLIM 1; SLIM; SLIM1) (FITC); anti-FHL1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human FHL1.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).

• Applicable Applications for anti-FHL1 antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:50-1:100; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the C-terminal region of human FHL1 (KLH).
• Conjugate: FITC
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer.

Western Blot (WB)

(Western blot analysis of FHL1 Antibody (C-term) in NIH-3T3 cell line lysates (35ug/lane). FHL1 (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human skeletal muscle reacted with FHL1 Antibody (C-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Flow Cytometry (FC/FACS)

(Flow cytometric analysis of widr cells using FHL1 Antibody (C-term)(bottom histogram) compared to a negative control cell (top histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Western Blot (WB)

(Western blot analysis of FHL1 (arrow) using rabbit polyclonal FHL1 Antibody (C-term). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the FHL1 gene.)

Product Categories/Family for anti-FHL1 antibody

Antibodies; Transcription Factors; Zinc (Ring) Finger

References

1. Gueneau, L., et.al., Am. J. Hum. Genet. 85(3), 338-353 (2009).

NCBI and Uniprot Product Information

• NCBI GI #: 21361122
• NCBI GeneID: 2273
• NCBI Accession #: NP_001440
• NCBI GenBank Nucleotide #: NM_001449.4
• UniProt Accession #: Q13642; O95212; Q13230; Q13645; Q5JXI7; Q5M7Y6; Q6IB30; Q9NZ40; Q9UKZ8; Q9Y630; B7Z5T4; B7Z793
• Molecular Weight: 33,579 Da
• NCBI Official Full Name: four and a half LIM domains protein 1 isoform 2
• NCBI Official Synonym Full Names: four and a half LIM domains 1
• NCBI Official Symbol: FHL1
• NCBI Official Synonym Symbols: KYOT; SLIM; FHL-1; FHL1A; FHL1B; FLH1A; SLIM1; XMPMA; 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