Anti-CCM2 Antibody (CAB6544)

Antibody Name:	Anti-CCM2 Antibody
Antibody SKU:	CAB6544
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human CCM2 (NP_113631.1).

Application:	WB IHC
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	MCF7

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human CCM2 (NP_113631.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	MEEE GKKG KKPG IVSP FKRV FLKG EKSR DKKA HEKV TERR PLHT VVLS LPER VEPD RLLS DYIE KEVK YLGQ LTSI PGYL NPSS RTEI LHFI DNAK RAHQ LPGH LTQE HDAV LSLS AYNV KLAW RDGE DIIL RVPI HDIA AVSY VRDD AAHL VVLK TAQD PGIS PSQS LCAE SSRG LSAG SLSE SAVG PVEA CCLV ILAA ESKV AAEE LCCL LGQV FQVV YTES TIDF LDRA IFDG ASTP THHL SLHS DDSS TKVD IKET YEVE ASTF CFPE SVDV GGAS PHSK TISE SELS ASAT ELLQ
Gene ID:	83605
Uniprot:	Q9BSQ5
Cellular Location:	Cytoplasm
Calculated MW:	39kDa/42kDa/48kDa/51kDa
Observed MW:	55kDa

Synonyms:

CCM2, C7orf22, OSM, PP10187

Background:

This gene encodes a scaffold protein that functions in the stress-activated p38 Mitogen-activated protein kinase (MAPK) signaling cascade. The protein interacts with SMAD specific E3 ubiquitin protein ligase 1 (also known as SMURF1) via a phosphotyrosine binding domain to promote RhoA degradation. The protein is required for normal cytoskeletal structure, cell-cell interactions, and lumen formation in endothelial cells. Mutations in this gene result in cerebral cavernous malformations. Multiple transcript variants encoding different isoforms have been found for this gene.

UniProt Protein Function:	CCM2: Component of the CCM signaling pathway which is a crucial regulator of heart and vessel formation and integrity. May act through the stabilization of endothelial cell junctions. May function as a scaffold protein for MAP2K3-MAP3K3 signaling. Seems to play a major role in the modulation of MAP3K3- dependent p38 activation induced by hyperosmotic shock. Defects in CCM2 are the cause of cerebral cavernous malformations type 2 (CCM2). Cerebral cavernous malformations (CCMs) are congenital vascular anomalies of the central nervous system that can result in hemorrhagic stroke, seizures, recurrent headaches, and focal neurologic deficits. CCMs have an incidence of 0.1%-0.5% in the general population and are usually present clinically during the 3rd to 5th decades of life. The lesions are characterized by grossly enlarged blood vessels consisting of a single layer of endothelium and without any intervening neural tissue, ranging in diameter from a few millimeters to several centimeters. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Motility/polarity/chemotaxis; Adaptor/scaffold Chromosomal Location of Human Ortholog: 7p13 Cellular Component: protein complex; cytoplasm Molecular Function:protein binding Biological Process: integrin-mediated signaling pathway; intercellular junction assembly and maintenance; pericardium development; venous blood vessel morphogenesis; in utero embryonic development; multicellular organism growth; stress-activated MAPK cascade; vasculogenesis; inner ear development; endothelial cell development Disease: Cerebral Cavernous Malformations 2
NCBI Summary:	This gene encodes a scaffold protein that functions in the stress-activated p38 Mitogen-activated protein kinase (MAPK) signaling cascade. The protein interacts with SMAD specific E3 ubiquitin protein ligase 1 (also known as SMURF1) via a phosphotyrosine binding domain to promote RhoA degradation. The protein is required for normal cytoskeletal structure, cell-cell interactions, and lumen formation in endothelial cells. Mutations in this gene result in cerebral cavernous malformations. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Nov 2009]
UniProt Code:	Q9BSQ5
NCBI GenInfo Identifier:	74733042
NCBI Gene ID:	83605
NCBI Accession:	Q9BSQ5.1
UniProt Secondary Accession:	Q9BSQ5,Q71RE5, Q8TAT4, A4D2L4, B3KUV0, D3DVL4, E9PDJ3 F5H0E1, F5H551,
UniProt Related Accession:	Q9BSQ5
Molecular Weight:	444
NCBI Full Name:	Malcavernin
NCBI Synonym Full Names:	cerebral cavernous malformation 2
NCBI Official Symbol:	CCM2
NCBI Official Synonym Symbols:	OSM; C7orf22; PP10187
NCBI Protein Information:	malcavernin; cerebral cavernous malformations 2 protein
UniProt Protein Name:	Malcavernin
UniProt Synonym Protein Names:	Cerebral cavernous malformations 2 protein
Protein Family:	Cerebral cavernous malformations protein
UniProt Gene Name:	CCM2
UniProt Entry Name:	CCM2_HUMAN

	Western blot analysis of extracts of MCF7 cells, using CCM2 antibody (CAB6544) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
	Immunohistochemistry of paraffin-embedded human liver using CCM2 antibody (CAB6544) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded rat heart using CCM2 antibody (CAB6544) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded mouse heart using CCM2 antibody (CAB6544) at dilution of 1:100 (40x lens).

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)