Alternative Name(s):

Cardiac LIM protein;Cysteine-rich protein 3 ;CRP3LIM domain protein, cardiacMuscle LIM protein

Species: (Organism)

Homo sapiens (Human)

Gene Names:

CSRP3

Tag info:

N-terminal 6xHis-SUMO-tagged

Target Protein AA Sequence:

MPNWGGGAKCGACEKTVYHAEEIQCNGRSFHKTCFHCMACRKALDSTTVAAHESEIYCKVCYGRRYGPKGIGYGQGAGCLSTDTGEHLGLQFQQSPKPARSVTTSNPSKFTAKFGESEKCPRCGKSVYAAEKVMGGGKPWHKTCFRCAICGKSLESTNVTDKDGELYCKVCYAKNFGPTGIGFGGLTQQVEKKE

Expression Region:

1-194aa

Subcellular Location:

Nucleus, Cytoplasm, Cytoplasm, cytoskeleton, Cytoplasm, myofibril, sarcomere, Z line, Cytoplasm, myofibril, sarcomere

Tissue Specificity:

Cardiac and slow-twitch skeletal muscles. Isoform 2 is expressed in striated muscle. Isoform 2 is specifically expressed at higher levels in patients with neuromuscular diseases, such as limb-girdle muscular dystrophy 2A (LGMD2A), Duchenne muscular dystrophy (DMD) and dermatomyositis (PubMed:24860983).

Protein Length:

Full Length

Pathway:

Mol. Weight:

37 kDa

Purity:

Greater than 90% as determined by SDS-PAGE.

Form:

Liquid or Lyophilized powder

Buffer:

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Research Areas:

Developmental Biology

Function:

Positive regulator of myogenesis. Acts as cofactor for myogenic bHLH transcription factors such as MYOD1, and probably MYOG and MYF6. Enhances the DNA-binding activity of the MYOD1

Involvement in disease:

Cardiomyopathy, dilated 1M (CMD1M); Cardiomyopathy, familial hypertrophic 12 (CMH12)

Relevance:

Positive regulator of myogenesis. Plays a crucial and specific role in the organization of cytosolic structures in cardiomyocytes. Could play a role in mechanical stretch sensing. May be a scaffold protein that promotes the assbly of interacting proteins at Z-line structures. It is essential for calcineurin anchorage to the Z line. Required for stress-induced calcineurin-NFAT activation .

Reconstitution:

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Protein Families:

Reference:

Beyond the sarcomere CSRP3 mutations cause hypertrophic cardiomyopathy.Geier C., Gehmlich K., Ehler E., Hassfeld S., Perrot A., Hayess K., Cardim N., Wenzel K., Erdmann B., Krackhardt F., Posch M.G., Osterziel K.J., Bublak A., Nagele H., Scheffold T., Dietz R., Chien K.R., Spuler S. , Furst D.O., Nurnberg P., Ozcelik C.Hum. Mol. Genet. 17:2753-2765(2008)