2wqa | pdb_00002wqa

COMPLEX OF TTR AND RBP4 AND OLEIC ACID

spinqualitylabelsall models 2wqa, resolution 2.85Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Structural highlights 2wqa is a 6 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance. Ligands: OLA, SO4 Related: 1rbp, 2g9k, 1dvy, 1eta, 2b9a, 1ttr, 1iii, 1dvt, 1bze, 1ijn, 1bzd, 1tt6, 1e3f, 1tlm, 2b77, 1f41, 1tyr, 1e5a, 1sok, 2pab, 1e4h, 2rox, 2b15, 1fhn, 1fh2, 1g1o, 2b16, 2f8i, 1iik, 1tta, 1bmz, 2b14, 1dvs, 1z7j, 1gko, 2flm, 2fbr, 2try, 1dvu, 1bz8, 1ttb, 1thc, 1qwh, 1dvz, 1tha, 1dvq, 1y1d, 1qab, 1u21, 1f86, 1soq, 2f7i, 1x7s, 1jyd, 1zcr, 1ict, 1ttc, 1dvx, 1bm7, 2roy, 1tz8, 1rlb, 1etb, 5ttr, 2g5u, 1f64, 2gab, 1x7t, 1jyj, 1tsh, 2trh, 2wq9, 1zd6 Resources: FirstGlance, OCA, RCSB, PDBsum Disease [TTHY_HUMAN] Defects in TTR are the cause of amyloidosis transthyretin-related (AMYL-TTR) [MIM:105210]. A hereditary generalized amyloidosis due to transthyretin amyloid deposition. Protein fibrils can form in different tissues leading to amyloid polyneuropathies, amyloidotic cardiomyopathy, carpal tunnel syndrome, systemic senile amyloidosis. The disease includes leptomeningeal amyloidosis that is characterized by primary involvement of the central nervous system. Neuropathologic examination shows amyloid in the walls of leptomeningeal vessels, in pia arachnoid, and subpial deposits. Some patients also develop vitreous amyloid deposition that leads to visual impairment (oculoleptomeningeal amyloidosis). Clinical features include seizures, stroke-like episodes, dementia, psychomotor deterioration, variable amyloid deposition in the vitreous humor. Defects in TTR are a cause of hyperthyroxinemia dystransthyretinemic euthyroidal (HTDE) [MIM:145680]. It is a condition characterized by elevation of total and free thyroxine in healthy, euthyroid persons without detectable binding protein abnormalities. Defects in TTR are a cause of carpal tunnel syndrome type 1 (CTS1) [MIM:115430]. It is a condition characterized by entrapment of the median nerve within the carpal tunnel. Symptoms include burning pain and paresthesias involving the ventral surface of the hand and fingers which may radiate proximally. Impairment of sensation in the distribution of the median nerve and thenar muscle atrophy may occur. This condition may be associated with repetitive occupational trauma, wrist injuries, amyloid neuropathies, rheumatoid arthritis. [RET4_HUMAN] Defects in RBP4 are a cause of retinol-binding protein deficiency (RBP deficiency) [MIM:180250]. This condition causes night vision problems. It produces a typical 'fundus xerophthalmicus', featuring a progressed atrophy of the retinal pigment epithelium. Function [TTHY_HUMAN] Thyroid hormone-binding protein. Probably transports thyroxine from the bloodstream to the brain. [RET4_HUMAN] Delivers retinol from the liver stores to the peripheral tissues. In plasma, the RBP-retinol complex interacts with transthyretin, this prevents its loss by filtration through the kidney glomeruli. Evolutionary Conservation Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf. See Also Retinol-binding protein Transthyretin

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