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Rabbit Anti-GCAP1/FITC Conjugated antibody
background:
intracellular stimulation of guanylate cyclase (GC) by calcium, a key event in the recovery of the dark state of rod photoreceptors after exposure to light, is mediated by guanylate cyclase-activating protein (GCAP1). GCAPs are calcium-The binding proteins belonging to the calmodulin superfamily. GCAP1 is a calcium-binding protein that stimulates synthesis of c-GMP in photoreceptors. GCAP1 is present in rod and cone photoreceptor outer segments where phototransduction occurs. In contrast to other calcium-binding proteins from the calmodulin superfamily, the calcium-free form of GCAP1 stimulates the effector enzyme. By molecular cloning of human and mouse GCAP cDNA, the known mammalian GCAPs are found to be more than 90% similar, consisting of 201 to 205 amino acids, and containing three identically conserved calcium-binding sites. A related protein, GCAP2, is detectable only in the retina and results from a gene duplication event.
Function:
Stimulates guanylyl cyclase 1 (GC1) when free calcium ions concentration is low and inhibits GC1 when free calcium ions concentration is elevated. This Ca(2+)-sensitive regulation of GC is a key event in recovery of the dark state of rod photoreceptors following light exposure.
Subcellular Location:
Membrane.
Tissue Specificity:
Retina; cone outer and inner segments, in particular, in disk membrane regions, and to a lesser extent rod inner and outer segments.
DISEASE:
Defects in GUCA1A are the cause of cone dystrophy type 3 (COD3) [MIM:602093]. COD3 is an autosomal dominant cone dystrophy. Cone dystrophies are retinal dystrophies characterized by progressive degeneration of the cone photoreceptors with preservation of rod function, as indicated by electroretinogram. However, some rod involvement may be present in some cone dystrophies, particularly at late stage. Affected individuals suffer from photophobia, loss of visual acuity, color vision and central visual field. Another sign is the absence of macular lesions for many years. Cone dystrophies are distinguished from the cone-rod dystrophies, in which some loss of peripheral vision also occurs.
Similarity:
Contains 4 EF-hand domains.
Database links:
Entrez Gene: 2978 Human
Omim: 600364 Human
SwissProt: P43080 Human
Unigene: 92858 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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