CCM-2

CAT: 0209-102-PA26Size: 200 µgDry Ice: NoHazardous: No
CAT#:0209-102-PA26Size:200 µg
Selected
PRICE
AVAILABILITY: InStock
24/48H Stock Items & 2 to 6 Weeks non Stock Items.
Product image 1
1 / 1
Description
Cerebral cavernous malformations (CCMs) are sporadically acquired or inherited vascular lesions of the central nervous system consisting of clusters of dilated thin-walled blood vessels that predispose individuals to seizures and stroke. Familial CCM is caused by mutations in KRIT1 (CCM1) or in malcavernin (CCM2) . The roles of the CCM proteins in the pathogenesis of the disorder remain largely unknown. It was shown that the CCM1 gene product, KRIT1, interacts with the CCM2 gene product, malcavernin. Analogous to the established interactions of CCM1 and beta1 integrin with ICAP1, the CCM1/CCM2 association is dependent upon the phosphotyrosine binding (PTB) domain of CCM2. A familial CCM2 missense mutation abrogates the CCM1/CCM2 interaction, suggesting that loss of this interaction may be critical in CCM pathogenesis. CCM2 and ICAP1 bound to CCM1 via their respective PTB domains differentially influence the subcellular localization of CCM1. The data indicate that the genetic heterogeneity observed in familial CCM may reflect mutation of different molecular members of a coordinated signaling complex.
Synonyms
CCM-2; malcavernin; cerebral cavernous malformation 2; OSM; C7orf22; PP10187
NCBI Gene ID
83605
UniProt
Q9BSQ5
Accession Number
NP_001025006.1
Accession Number mRNA
NM_001029835.2
Chromosomal Location
7p13
Reactivity
Anti-Human
Cross Reactivity
Human
Target Antigen
Recombinant human CCM2 (RT #300-055)
Clone
Rabbit IgG
Applications
WB, IF
Purification Method
Protein A purified
Assay Protocol
Centrifuge vial prior to opening. Reconstitute in sterile water to a concentration of 0.1-1.0 mg/ml.
Bioactivity
Western Blot: Use 1-5 µg/ml
Form
Lyophilized
Buffer
0.5X PBS, pH 7.2
Reconstitution
Water
Storage Conditions
The lyophilized antibody is stable for at least 2 years at -20°C. After sterile reconstitution the antibody is stable at 2-8°C for up to 6 months. Frozen aliquots are stable for at least 6 months when stored at -20°C. Addition of a carrier protein or 50% glycerol is recommended for frozen aliquots.
Host or Source
Rabbit

Related Products

CatalogName
102-PA27CCM-3
102-PA25CCM-1
300-056CCM-3
300-054CCM-1
102-PA25SCCM-1
102-PA27SCCM-3
300-050Angiopoietin-2 / Ang-2
300-050-L500Angiopoietin-2 / Ang-2
300-049Angiopoietin-2 / Ang-2
101-M102Angiopoietin-2 / Ang-2

Alternative Products

CatalogName
MF-0114284-01β-CCM
MBS691545-01Human CCM-2
102-PA26Rabbit Anti-Human CCM-2
MBS691618-01Human CCM-3
MBS692224-01Human CCM-1
ABC-TC0696MOG-G-CCM
102-PA27Rabbit Anti-Human CCM-3
RWT-590MOG-G-CCM Cell Line
102-PA25Rabbit Anti-Human CCM-1
MBS9016204Rabbit anti-Caenorhabditis elegans ccm-3 Polyclonal Antibody

Popular Products