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About This Item
UNSPSC Code:
12352203
NACRES:
NA.41
biological source
mouse
Quality Level
conjugate
unconjugated
antibody form
IgG fraction of antiserum
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
species reactivity
human
technique(s)
western blot: 1 μg/mL
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... CCM2(83605)
General description
Cerebral cavernous malformation 2 (CCM2) is an adaptor protein consisting of two domains. Structurally, it has two domains, N-terminal phosphotyrosine-binding (PTB) domain and an independent domain named as Karet domain at the C-terminal end. It is mapped on human chromosome 7p. Its phosphotyrosine-binding (PTB) domain has the ability to bind proteins. It is expressed in vascular endothelium and neuroglial precursor cells during development and also observed in arterial endothelium, neurons, and some of the glial cells in adult neocortex.
Immunogen
CCM2 (NP_113631.1, 1 a.a. ~ 444 a.a) full-length human protein.
Sequence
MEEEGKKGKKPGIVSPFKRVFLKGEKSRDKKAHEKVTERRPLHTVVLSLPERVEPDRLLSDYIEKEVKYLGQLTSIPGYLNPSSRTEILHFIDNAKRAHQLPGHLTQEHDAVLSLSAYNVKLAWRDGEDIILRVPIHDIAAVSYVRDDAAHLVVLKTAQDPGISPSQSLCAESSRGLSAGSLSESAVGPVEACCLVILAAESKVAAEELCCLLGQVFQVVYTESTIDFLDRAIFDGASTPTHHLSLHSDDSSTKVDIKETYEVEASTFCFPESVDVGGASPHSKTISESELSASATELLQDYMLTLRTKLSSQEIQQFAALLHEYRNGASIHEFCINLRQLYGDSRKFLLLGLRPFIPEKDSQHFENFLETIGVKDGRGIITDSFGRHRRALSTTSSSTTNGNRATGSSDDRSAPSEGDEWDRMISDISSDIEALGCSMDQDSA
Sequence
MEEEGKKGKKPGIVSPFKRVFLKGEKSRDKKAHEKVTERRPLHTVVLSLPERVEPDRLLSDYIEKEVKYLGQLTSIPGYLNPSSRTEILHFIDNAKRAHQLPGHLTQEHDAVLSLSAYNVKLAWRDGEDIILRVPIHDIAAVSYVRDDAAHLVVLKTAQDPGISPSQSLCAESSRGLSAGSLSESAVGPVEACCLVILAAESKVAAEELCCLLGQVFQVVYTESTIDFLDRAIFDGASTPTHHLSLHSDDSSTKVDIKETYEVEASTFCFPESVDVGGASPHSKTISESELSASATELLQDYMLTLRTKLSSQEIQQFAALLHEYRNGASIHEFCINLRQLYGDSRKFLLLGLRPFIPEKDSQHFENFLETIGVKDGRGIITDSFGRHRRALSTTSSSTTNGNRATGSSDDRSAPSEGDEWDRMISDISSDIEALGCSMDQDSA
Biochem/physiol Actions
Cerebral cavernous malformation 2 (CCM2) is associated with several cellular pathways. It mainly behaves as a scaffold protein in the activation of GTPase Rac-dependent p38 mitogen-activated protein kinase (MAPK) during hyperosmotic stress. It also participates in vascular integrity. CCM2 may play a role in vasculogenesis and angiogenesis during the development of the brain. Mutation in the gene is associated with familial cerebral cavernous malformations.
Physical form
Solution in phosphate buffered saline, pH 7.4
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Storage Class Code
10 - Combustible liquids
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Regulatory Information
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CCM2-CCM3 interaction stabilizes their protein expression and permits endothelial network formation.
Kyle M Draheim et al.
The Journal of cell biology, 208(7), 987-1001 (2015-04-01)
Mutations in the essential adaptor proteins CCM2 or CCM3 lead to cerebral cavernous malformations (CCM), vascular lesions that most frequently occur in the brain and are strongly associated with hemorrhagic stroke, seizures, and other neurological disorders. CCM2 binds CCM3, but
Gamze Tanriover et al.
International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 29(5), 509-514 (2011-05-17)
Cerebral cavernous malformation (CCM) is one of the most common types of vascular malformations of the central nervous system, affecting nearly one in 200 people. CCM lesions are characterized by grossly dilated vascular channels lined by a single layer of
Rosalia D'Angelo et al.
Gene, 519(1), 202-207 (2012-09-25)
Cerebral cavernous malformations (CCMs; OMIM 116860) are vascular anomalies mostly located in the central nervous system (CNS) and occasionally within the skin and retina. Main clinical manifestations are seizure, hemorrhage, recurrent headaches, focal neurological deficits and epileptic attacks. The CCMs
C Denier et al.
American journal of human genetics, 74(2), 326-337 (2004-01-24)
Cerebral cavernous malformations (CCM) are hamartomatous vascular malformations characterized by abnormally enlarged capillary cavities without intervening brain parenchyma. They cause seizures and focal neurological deficits due to cerebral hemorrhages. CCM loci have already been assigned to chromosomes 7q (CCM1), 7p
Xiaoyan Wang et al.
Acta crystallographica. Section F, Structural biology and crystallization communications, 68(Pt 6), 683-686 (2012-06-12)
Cerebral cavernous malformation 2 (CCM2) is a novel two-domain adaptor protein which participates in multiple cellular signalling pathways. Loss-of-function mutations in the gene encoding CCM2 are the cause of common human vascular lesions called cerebral cavernous malformations. Here, the purification
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