Catalog No: ARP34378_T100
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ARP34378_T100
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Datasheets/ManualsPrintable datasheet for anti-FHL1 (ARP34378_T100) antibody
Product Info
Tested Species ReactivityHuman, Mouse
Predicted Species ReactivityHuman, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Sheep
Product FormatLiquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
ClonalityPolyclonal
HostRabbit
ApplicationIHC, WB
Reconstitution and StorageFor 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.
ImmunogenThe immunogen is a synthetic peptide directed towards the C terminal region of human FHL1
PurificationProtein A purified
Predicted Homology Based on Immunogen SequenceCow: 100%; Dog: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 93%; Rat: 100%; Sheep: 100%
Peptide SequenceSynthetic peptide located within the following region: YYCVDCYKNFVAKKCAGCKNPITGFGKGSSVVAYEGQSWHDYCFHCKKCS
Concentration1.0 mg/ml
Blocking PeptideFor anti-FHL1 (ARP34378_T100) antibody is Catalog # AAP34378 (Previous Catalog # AAPY00220)
ReferenceMcGrath,M.J., et al., (2003) Am. J. Physiol., Cell Physiol. 285 (6), C1513-C1526
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

Description
Gene SymbolFHL1
Gene Full NameFour and a half LIM domains 1
Alias SymbolsKYOT, SLIM, FCMSU, FHL-1, FHL1A, FHL1B, FLH1A, SLIM1, XMPMA, RBMX1A, RBMX1B, SLIM-1, SLIMMER
NCBI Gene Id2273
Protein NameFour and a half LIM domains protein 1
Description of TargetLIM 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.
Uniprot IDQ6IB30
Protein Accession #NP_001440
Nucleotide Accession #NM_001449
Protein Size (# AA)280
Molecular Weight32kDa
Protein InteractionsSUMO2; 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;
  1. What is the species homology for "FHL1 Antibody - C-terminal region (ARP34378_T100)"?

    The tested species reactivity for this item is "Human, Mouse". This antibody is predicted to have homology to "Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Sheep".

  2. How long will it take to receive "FHL1 Antibody - C-terminal region (ARP34378_T100)"?

    This item is available "Domestic: within 1-2 days delivery | International: 1-2 days".

  3. What buffer format is "FHL1 Antibody - C-terminal region (ARP34378_T100)" provided in?

    This item is provided in "Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.".
    Additional format options may be available. For more information please contact info@avivasysbio.com.

  4. What are other names for "FHL1 Antibody - C-terminal region (ARP34378_T100)"?

    This target may also be called "KYOT, SLIM, FCMSU, FHL-1, FHL1A, FHL1B, FLH1A, SLIM1, XMPMA, RBMX1A, RBMX1B, SLIM-1, SLIMMER" in publications.

  5. What is the shipping cost for "FHL1 Antibody - C-terminal region (ARP34378_T100)"?

    The shipping cost for this item is $40 within the US. Please contact us for specific shipping prices for international orders.

  6. What is the guarantee for "FHL1 Antibody - C-terminal region (ARP34378_T100)"?

    All Aviva products have been through rigorous validations and carry 100% satisfaction guarantee.

  7. Can I get bulk pricing for "FHL1 Antibody - C-terminal region (ARP34378_T100)"?

    You can get bulk pricing for this item by going here.

  8. What is the molecular weight of the protein?

    The molecular weight reported by Uniprot for this item is "32kDa".
    Please note observed molecular weights in western blot applications may differ depending on a variety of protein characteristics.

  9. What protocols are available for "FHL1 Antibody - C-terminal region (ARP34378_T100)"?

    We may have detailed protocol data avaialble for this item. To learn more, please view the "Protocols & Data" tab on the product page.

  10. What are positive controls for "FHL1"?

    We have listed RNA Seq and gene expression data in the "Target Info" tab. You may be able to find adequate positive controls there.

  11. What are negative controls for "FHL1"?

    We have listed RNA Seq and gene expression data in the "Target Info" tab. You may be able to find adequate positive controls there.

  12. What other proteins interact with "FHL1"?

    This protein has been reported to interact with "Protein Interactions". Please view the "Related Categories" tab on the product page for more information.

  13. What biological processes are associated with "FHL1"?

    This protein has been associated with "Biological Processes". Please view the "Related Categories" tab on the product page for more information.

  14. What cellular components are associated with "FHL1"?

    This protein has been associated with "Cellular Components". Please view the "Related Categories" tab on the product page for more information.

  15. What protein functions are associated with "FHL1"?

    This protein has been associated with "Protein Functions". Please view the "Related Categories" tab on the product page for more information.

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