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FHL1 recombinant protein :: Four and a half LIM domains protein 1 (FHL1) Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS1476116
SDS-PAGE
Unit / Price
0.5 mg (E-Coli)  /  $1,080 +1 FREE 8GB USB
0.05 mg (Baculovirus)  /  $1,080 +1 FREE 8GB USB
0.5 mg (Yeast)  /  $1,315 +1 FREE 8GB USB
0.05 mg (Mammalian-Cell)  /  $1,315 +1 FREE 8GB USB
1 mg (E-Coli)  /  $1,700 +2 FREE 8GB USB
0.1 mg (Baculovirus)  /  $1,700 +2 FREE 8GB USB
1 mg (Yeast)  /  $2,165 +2 FREE 8GB USB
0.1 mg (Mammalian-Cell)  /  $2,165 +2 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Four and a half LIM domains protein 1 (FHL1), Recombinant Protein

 Also Known As   

Recombinant Human Four and a half LIM domains protein 1 (FHL1)

 Product Synonym Names    Four and a half LIM domains protein 1; FHL-1; Skeletal muscle LIM-protein 1; SLIM; SLIM-1
 Product Gene Name   

FHL1 recombinant protein

[Similar Products]
 Product Synonym Gene Name    FHL1; SLIM1 [Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 MBS1476116 COA    COA PDF
 Sequence Positions    2-323
 Sequence    AEKFDCHYC RDPLQGKKYV QKDGHHCCLK CFDKFCANTC VECRKPIGAD SKEVHYKNRF WHDTCFRCAK CLHPLANETF VAKDNKILCN KCTTREDSPK CKGCFKAIVA GDQNVEYKGT VWHKDCFTCS NCKQVIGTGS FFPKGEDFYC VTCHETKFAK HCVKCNKAIT SGGITYQDQP WHADCFVCVT CSKKLAGQRF TAVEDQYYCV DCYKNFVAKK CAGCKNPITG KRTVSRVSHP VSKARKPPVC HGKRLPLTLF PSANLRGRHP GGERTCPSWV VVLYRKNRSL AAPRGPGLVK APVWWPMKDN PGTTTASTAK NAP
 OMIM    300718
 3D Structure    ModBase 3D Structure for Q13642
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 Host    E Coli or Yeast or Baculovirus or Mammalian Cell
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 Purity/Purification    >90%
 Form/Format    Liquid containing glycerol; lyophilization may be available upon request.
 Tag Information    This protein contains an N-terminal tag and may also contain a C-terminal Myc-tag. N-terminal host tags may vary (His, His-SUMO, His-B2M, GST). Tag types are determined by various factors including tag-protein stability and, therefore, are subject to change; please inquire for tag information.
 Sterility    Sterile filter available upon request.
 Endotoxin    Low endotoxin available upon request.
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 Species    Homo sapiens (Human)
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 Preparation and Storage    Store at -20 degrees C. For long-term storage, store at -20 degrees C or -80 degrees C. Store working aliquots at 4 degrees C for up to one week. Repeated freezing and thawing is not recommended.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of FHL1 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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 SDS-PAGE of FHL1 recombinant protein    Recombinant Human Four and a half LIM domains protein 1(FHL1)
His-tagged
E.coli derived
Endotoxin Level: untreated
FHL1 recombinant protein SDS-PAGE image
(Note: Representative image, actual molecular weight may vary depending on Tag type and expression host)
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NCBI/Uniprot data below describe general gene information for FHL1. It may not necessarily be applicable to this product.
 NCBI GI #    228480205
 NCBI GeneID    2273
 NCBI Accession #    NP_001153171.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001159699.1 [Other Products]
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 UniProt Primary Accession #    Q13642 [Other Products]
 UniProt Secondary Accession #    O95212; Q13230; Q13645; Q5JXI7; Q5M7Y6; Q6IB30; Q9NZ40; Q9UKZ8; Q9Y630; B7Z5T4; B7Z793 [Other Products]
 UniProt Related Accession #    Q13642 [Other Products]
 Molecular Weight    33,579 Da
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 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 [Similar Products]
 NCBI Official Synonym Symbols   
KYOT; SLIM; FHL-1; FHL1A; FHL1B; FLH1A; SLIM1; XMPMA; SLIM-1; SLIMMER
[Similar Products]
 NCBI Protein Information    four and a half LIM domains protein 1; LIM protein SLIMMER; skeletal muscle LIM-protein 1; four-and-a-half Lin11, Isl-1 and Mec-3 domains 1
 UniProt Protein Name    Four and a half LIM domains protein 1
 UniProt Synonym Protein Names   
Skeletal muscle LIM-protein 1; SLIM; SLIM-1
 Protein Family    Fork head transcription factor
 UniProt Gene Name    FHL1 [Similar Products]
 UniProt Synonym Gene Names    SLIM1; FHL-1; SLIM; SLIM-1 [Similar Products]
 UniProt Entry Name    FHL1_HUMAN
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 NCBI Summary for FHL1    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]
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 UniProt Comments for FHL1    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: organ morphogenesis; muscle development; 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
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 Research Articles on FHL1    1. Data indicate that four-and-a-half LIM domain 1 gene FHL1 mutation causing isolated hypertrophic cardiomyopathy with X-chromosomal inheritance.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with FHL1 recombinant proteinPathways associated with FHL1 recombinant protein
 Reference Product  PubMed Publications
 FHL2 recombinant protein  >15 publications with FHL1 and FHL2
 RBPJ recombinant protein  >5 publications with FHL1 and RBPJ
 RING1 recombinant protein  >3 publications with FHL1 and RING1
 SRF recombinant protein  >1 publications with FHL1 and SRF
 Products by Pathway  Pathway Diagram
 Delta-Notch Signaling Pathway antibodies  Delta-Notch Signaling Pathway Diagram
Diseases associated with FHL1 recombinant proteinOrgans/Tissues associated with FHL1 recombinant protein
 Disease Name  Pubmed Publications
 Muscular Diseases Antibodies  >19 publications with FHL1 and Muscular Diseases
 Nervous System Diseases Antibodies  >18 publications with FHL1 and Nervous System Diseases
 Cardiovascular Diseases Antibodies  >16 publications with FHL1 and Cardiovascular Diseases
 Hypertrophy Antibodies  >13 publications with FHL1 and Hypertrophy
 Neoplasms Antibodies  >12 publications with FHL1 and Neoplasms
 Genetic Diseases, Inborn Antibodies  >12 publications with FHL1 and Genetic Diseases, Inborn
 MYOPATHY, X-LINKED, WITH POSTURAL MUSCLE ATROPHY Antibodies  >6 publications with FHL1 and MYOPATHY, X-LINKED, WITH POSTURAL MUSCLE ATROPHY
 Skin Diseases Antibodies  >5 publications with FHL1 and Skin Diseases
 Leukemia Antibodies  >4 publications with FHL1 and Leukemia
 Heart Failure Antibodies  >4 publications with FHL1 and Heart Failure
 Organ/Tissue Name  Pubmed Publications
 Muscle Antibodies  >87 publications with FHL1 and Muscle
 Heart Antibodies  >25 publications with FHL1 and Heart
 Kidney Antibodies  >8 publications with FHL1 and Kidney
 Lung Antibodies  >6 publications with FHL1 and Lung
 Brain Antibodies  >6 publications with FHL1 and Brain
 Liver Antibodies  >5 publications with FHL1 and Liver
 Prostate Antibodies  >4 publications with FHL1 and Prostate
 Vascular Antibodies  >4 publications with FHL1 and Vascular
 Embryonic Tissue Antibodies  >4 publications with FHL1 and Embryonic Tissue
 Intestine Antibodies  >3 publications with FHL1 and Intestine
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