FHL1 Antibody - C-terminal region (ARP34378_T100)

Data Sheet
 
Product Number ARP34378_T100
Product Page www.avivasysbio.com/fhl1-antibody-c-terminal-region-arp34378-t100.html
Name FHL1 Antibody - C-terminal region (ARP34378_T100)
Protein Size (# AA) 280 amino acids
Molecular Weight 32kDa
NCBI Gene Id 2273
Host Rabbit
Clonality Polyclonal
Concentration 1.0 mg/ml
Gene Full Name Four and a half LIM domains 1
Description
Alias Symbols KYOT, SLIM, FCMSU, FHL-1, FHL1A, FHL1B, FLH1A, SLIM1, XMPMA, RBMX1A, RBMX1B, SLIM-1, SLIMMER
Peptide Sequence Synthetic peptide located within the following region: YYCVDCYKNFVAKKCAGCKNPITGFGKGSSVVAYEGQSWHDYCFHCKKCS
Product Format Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Reference McGrath,M.J., et al., (2003) Am. J. Physiol., Cell Physiol. 285 (6), C1513-C1526
Description of Target LIM proteins, named for 'LIN11, ISL1, and MEC3,' are defined by the possession of a highly conserved double zinc finger motif called the LIM domain. FHL1 may play an important role during the early stages of skeletal muscle differentiation, specifically in alpha5beta1-integrin-mediated signaling pathways.
Protein Interactions SUMO2; UBC; SRPK1; SMAD4; SMAD3; SMAD2; LMNA; CSNK1D; NRIP1; ESR1; HIVEP3; SMURF1; UBE2E2; STAT4; RING1; RBPJ; FHL1; KCNA5; PRNP; USP15; HHV8GK18_gp81; SP1; TXNIP; DEAF1; PDE4DIP; AKAP12; EED; HES1; DBN1; CBX4; FHL2; SRF; MYBPC1;
Reconstitution and Storage For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
Datasheets/Manuals Printable datasheet for anti-FHL1 (ARP34378_T100) antibody
Blocking Peptide For anti-FHL1 (ARP34378_T100) antibody is Catalog # AAP34378 (Previous Catalog # AAPY00220)
Immunogen The immunogen is a synthetic peptide directed towards the C terminal region of human FHL1
Uniprot ID Q6IB30
Protein Name Four and a half LIM domains protein 1
Publications

Cardiomyopathy and altered integrin-actin signaling in Fhl1 mutant female mice. Hum Mol Genet. 28, 209-219 (2019). 30260394

Dong, R. et al. Cells with dendritic cell morphology and immunophenotype, binuclear morphology, and immunosuppressive function in dendritic cell cultures. Cell. Immunol. 272, 1-10 (2011). 22053194

Exome Sequencing Identified a Splice Site Mutation in FHL1 that Causes Uruguay Syndrome, an X-Linked Disorder With Skeletal Muscle Hypertrophy and Premature Cardiac Death. Circ Cardiovasc Genet. 9, 130-5 (2016). 26933038

Fhl1 W122S causes loss of protein function and late-onset mild myopathy. Hum Mol Genet. 24, 714-26 (2015). 25274776

Fujii, T. et al. A case of adult-onset reducing body myopathy presenting a novel clinical feature, asymmetrical involvement of the sternocleidomastoid and trapezius muscles. J. Neurol. Sci. 343, 206-10 (2014). 24928078

Gueneau, L. et al. Mutations of the FHL1 gene cause Emery-Dreifuss muscular dystrophy. Am. J. Hum. Genet. 85, 338-53 (2009). 19716112

Immunohistochemistry on a panel of Emery-Dreifuss muscular dystrophy samples reveals nuclear envelope proteins as inconsistent markers for pathology. Neuromuscul. Disord. 27, 338-351 (2017). 28214269

Koike, K. et al. High prevalence of epigenetic inactivation of the human four and a half LIM domains 1 gene in human oral cancer. Int. J. Oncol. 42, 141-50 (2013). 23123766

Loss of FHL1 induces an age-dependent skeletal muscle myopathy associated with myofibrillar and intermyofibrillar disorganization in mice. Hum Mol Genet. 23, 209-25 (2014). 23975679

Quinzii, C. M. et al. X-linked dominant scapuloperoneal myopathy is due to a mutation in the gene encoding four-and-a-half-LIM protein 1. Am. J. Hum. Genet. 82, 208-13 (2008). 18179901

Schoser, B. et al. Consequences of mutations within the C terminus of the FHL1 gene. Neurology 73, 543-51 (2009). 19687455

Sharma, P., Shathasivam, T., Ignatchenko, V., Kislinger, T. & Gramolini, A. O. Identification of an FHL1 protein complex containing ACTN1, ACTN4, and PDLIM1 using affinity purifications and MS-based protein-protein interaction analysis. Mol. Biosyst. 7, 1185-96 (2011). 21246116

Windpassinger, C. et al. An X-linked myopathy with postural muscle atrophy and generalized hypertrophy, termed XMPMA, is caused by mutations in FHL1. Am. J. Hum. Genet. 82, 88-99 (2008). 18179888

Protein Accession # NP_001440
Purification Protein A purified
Nucleotide Accession # NM_001449
Tested Species Reactivity Human, Mouse
Gene Symbol FHL1
Predicted Species Reactivity Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Sheep
Application IHC, WB
Predicted Homology Based on Immunogen Sequence Cow: 100%; Dog: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 93%; Rat: 100%; Sheep: 100%
Image 1
Mouse Skeletal Muscle
Host: Mouse
Target Name: FHL1
Sample Tissue: Mouse Skeletal Muscle
Antibody Dilution: 1ug/ml
Image 2
Human Fetal Heart
Host: Rabbit
Target Name: FHL1
Sample Type: Human Fetal Heart
Lane A: Primary Antibody
Lane B: Primary Antibody + Blocking Peptide
Primary Antibody Concentration: 1ug/ml
Peptide Concentration: 5ug/ml
Lysate Quantity: 25ug/lane/lane
Gel Concentration: 0.12
Image 3
Human kidney
Human kidney
Image 4
Human Muscle
WB Suggested Anti-FHL1 Antibody Titration: 0.1ug/ml
ELISA Titer: 1:312500
Positive Control: Human Muscle
Image 5
Human heart muscle, Human liver
Host: Rabbit
Target: FHL1
Positive control (+): Human heart muscle
Negative control (-): Human liver (LI)
Antibody concentration: 1ug/ml
 

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