1B4R

PKD DOMAIN 1 FROM HUMAN POLYCYSTEIN-1

Summary for 1B4R

• Entry DOI: 10.2210/pdb1b4r/pdb
• NMR Information: BMRB: 4478
• Descriptor: PROTEIN (PKD1_HUMAN) (1 entity in total)
• Functional Keywords: pkd domain 1 from human polycystein-1, polycystin (precursor), membrane protein
• Biological source: Homo sapiens (human)
• Cellular location: Membrane; Multi-pass membrane protein (Potential): P98161
• Total number of polymer chains: 1
• Total formula weight: 7963.91

Authors

Bycroft, M. (deposition date: 1998-12-28, release date: 1999-01-06, Last modification date: 2024-05-22)

Primary citation

Bycroft, M.,Bateman, A.,Clarke, J.,Hamill, S.J.,Sandford, R.,Thomas, R.L.,Chothia, C.
The structure of a PKD domain from polycystin-1: implications for polycystic kidney disease.
EMBO J., 18:297-305, 1999

PubMed Abstract: Most cases of autosomal dominant polycystic kidney disease (ADPKD) are the result of mutations in the PKD1 gene. The PKD1 gene codes for a large cell-surface glycoprotein, polycystin-1, of unknown function, which, based on its predicted domain structure, may be involved in protein-protein and protein-carbohydrate interactions. Approximately 30% of polycystin-1 consists of 16 copies of a novel protein module called the PKD domain. Here we show that this domain has a beta-sandwich fold. Although this fold is common to a number of cell-surface modules, the PKD domain represents a distinct protein family. The tenth PKD domain of human and Fugu polycystin-1 show extensive conservation of surface residues suggesting that this region could be a ligand-binding site. This structure will allow the likely effects of missense mutations in a large part of the PKD1 gene to be determined.

PubMed: 9889186
DOI: 10.1093/emboj/18.2.297

Experimental method

SOLUTION NMR