Description
Antibody Name: | IMPG2 Antibody (PACO61546) |
Antibody SKU: | PACO61546 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, IF |
Recommended Dilutions: | ELISA:1:2000-1:10000, IF:1:50-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Interphotoreceptor matrix proteoglycan 2 protein (616-759AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Immunofluorescence staining of Hela cells with PACO61546 at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
Background: | Chondroitin sulfate- and hyaluronan-binding proteoglycan involved in the organization of interphotoreceptor matrix; may participate in the maturation and maintenance of the light-sensitive photoreceptor outer segment. Binds heparin. |
Synonyms: | Interphotoreceptor matrix proteoglycan 2, Interphotoreceptor matrix proteoglycan of 200 kDa, IPM 200, Sialoprotein associated with cones and rods proteoglycan, Spacrcan, IMPG2, IPM200 |
UniProt Protein Function: | IMPG2: Chondroitin sulfate- and hyaluronan-binding proteoglycan involved in the organization of interphotoreceptor matrix; may participate in the maturation and maintenance of the light- sensitive photoreceptor outer segment. Binds heparin. Defects in IMPG2 are the cause of retinitis pigmentosa type 56 (RP56). RP56 is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. Defects in IMPG2 are the cause of maculopathy IMPG2- related (MACLP-IMPG2). MACLP-IMPG2 is a mild maculopathy characterized by full-field electroretinogram responses within normal limits, normal color vision, elevation of the photoreceptor layer in the foveal region and mild nuclear sclerosis.Protein type: Extracellular matrix; Membrane protein, integralChromosomal Location of Human Ortholog: 3q12.2-q12.3Cellular Component: proteinaceous extracellular matrix; integral to membrane; receptor complexMolecular Function: heparin binding; extracellular matrix structural constituent; hyaluronic acid bindingBiological Process: visual perceptionDisease: Retinitis Pigmentosa 56; Macular Dystrophy, Vitelliform, 5 |
UniProt Protein Details: | |
NCBI Summary: | The protein encoded by this gene binds chondroitin sulfate and hyaluronan and is a proteoglycan. The encoded protein plays a role in the organization of the interphotoreceptor matrix and may promote the growth and maintenance of the light-sensitive photoreceptor outer segment. Defects in this gene are a cause of retinitis pigmentosa type 56 and maculopathy, IMPG2-related.[provided by RefSeq, Mar 2011] |
UniProt Code: | Q9BZV3 |
NCBI GenInfo Identifier: | 57242793 |
NCBI Gene ID: | 50939 |
NCBI Accession: | NP_057331.2 |
UniProt Secondary Accession: | Q9BZV3,Q9UKD4, Q9UKK5, A8MWT5 |
UniProt Related Accession: | Q9BZV3 |
Molecular Weight: | 138,621 Da |
NCBI Full Name: | interphotoreceptor matrix proteoglycan 2 |
NCBI Synonym Full Names: | interphotoreceptor matrix proteoglycan 2 |
NCBI Official Symbol: | IMPG2 |
NCBI Official Synonym Symbols: | RP56; IPM200; SPACRCAN |
NCBI Protein Information: | interphotoreceptor matrix proteoglycan 2; IPM 200; interphotoreceptor matrix proteoglycan IPM 200; interphotoreceptor matrix proteoglycan of 200 kDa; sialoprotein associated with cones and rods proteoglycan |
UniProt Protein Name: | Interphotoreceptor matrix proteoglycan 2 |
UniProt Synonym Protein Names: | Interphotoreceptor matrix proteoglycan of 200 kDa; IPM 200; Sialoprotein associated with cones and rods proteoglycan; Spacrcan |
Protein Family: | Interphotoreceptor matrix proteoglycan |
UniProt Gene Name: | IMPG2 |
UniProt Entry Name: | IMPG2_HUMAN |