Recombinant Human FLNC protein (His tag)

Specifications

Catalog number

PDEH100853-01

Size / count

20 µg

Dry ice

No

Hazardous

No

Discontinued

No

Background

FLNC is a muscle-specific filamin, which plays a central role in muscle cells, probably by functioning as a large actin-cross-linking protein. May be involved in reorganizing the actin cytoskeleton in response to signaling events, and may also display structural functions at the Z-disks in muscle cells. Defects in FLNC are the cause of autosomal dominant filaminopathy. Myofibrillar myopathy (MFM) is a neuromuscular disorder, usually with an adult onset, characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations. Autosomal dominant filaminopathy is a form of MFM characterized by morphological features of MFM and clinical features of a limb-girdle myopathy. A heterozygous nonsense mutation which segregates with the disease, has been identified in the FLNC gene.

Abbreviation

FLNC

Synonyms

ABPL; FIlamin 2; FLN2; FLNC; Filamin C; Filamin C

UniProt

Q14315

Accession Number

Q14315

Expression System

E.coli

Tag

N-His

Sequence

Thr 2519-Pro 2725

Field of Research

Signal transduction

Endotoxin

< 10 EU/mg of the protein as determined by the LAL method.

Purity

> 95% as determined by reducing SDS-PAGE.

Bioactivity

Not validated for activity

Reconstitution

It is recommended that sterile water be added to the vial to prepare a stock solution of 0.5 mg/mL. Concentration is measured by UV-Vis.

Shipping Conditions

This product is provided as lyophilized powder which is shipped with ice packs.

Storage Conditions

Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.

Calculated Molecular Weight

22.7 kDa

Observed Molecular Weight

28 kDa

Species

Human