ID PRIO_HUMAN STANDARD; PRT; 253 AA.
AC P04156;
DE MAJOR PRION PROTEIN PRECURSOR (PRP) (PRP27-30) (PRP33-35C) (ASCR).
OS Homo sapiens (Human).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
OC Mammalia; Eutheria; Primates; Catarrhini; Hominidae; Homo.
OX NCBI_TaxID=9606;
RN [1]
RP SEQUENCE FROM N.A.
RX MEDLINE=86300093 [NCBI, ExPASy, Israel, Japan]; PubMed=3755672;
RA Kretzschmar H.A., Stowring L.E., Westaway D., Stubblebine W.H., Prusiner S.B., Dearmond S.J.
RT "Molecular cloning of a human prion protein cDNA.";
RL DNA 5:315-324(1986).
RN [6]
RP STRUCTURE BY NMR OF 23-231.
RX MEDLINE=97424376 [NCBI, ExPASy, Israel, Japan]; PubMed=9280298;
RA Riek R., Hornemann S., Wider G., Glockshuber R., Wuethrich K.;
RT "NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231).";
RL FEBS Lett. 413:282-288(1997).
CC -!- FUNCTION: THE FUNCTION OF PRP IS NOT KNOWN. PRP IS ENCODED IN THE HOST GENOME AND IS
CC EXPRESSED BOTH IN NORMAL AND INFECTED CELLS.
CC -!- SUBUNIT: PRP HAS A TENDENCY TO AGGREGATE YIELDING POLYMERS CALLED "RODS".
CC -!- SUBCELLULAR LOCATION: ATTACHED TO THE MEMBRANE BY A GPI-ANCHOR.
CC -!- DISEASE: PRP IS FOUND IN HIGH QUANTITY IN THE BRAIN OF HUMANS AND ANIMALS INFECTED WITH CC NEURODEGENERATIVE DISEASES KNOWN AS TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES OR PRION CC DISEASES, LIKE: CREUTZFELDT-JAKOB DISEASE (CJD), GERS
CC FATAL FAMILIAL INSOMNIA (FFI) AND KURU IN HUMANS; SCRAPIE IN SHEEP AND GOAT; BOVINE
CC SPONGIFORM ENCEPHALOPATHY (BSE) IN CATTLE; TRANSMISSIBLE MINK ENCEPHALOPATHY (TME);
CC CHRONIC WASTING DISEASE (CWD) OF MULE DEER AND ELK; FELINE SPONGIFORM ENCEPHALOPATHY
CC (FSE) IN CATS AND EXOTIC UNGULATE ENCEPHALOPATHY(EUE) IN NYALA AND GREATER KUDU. THE
CC PRION DISEASES ILLUSTRATE THREE MANIFESTATIONS OF CNS DEGENERATION: (1) INFECTIOUS (2)
CC SPORADIC AND (3) DOMINANTLY INHERITED FORMS. TME, CWD, BSE, FSE, EUE ARE ALL THOUGHT TO CC OCCUR AFTER CONSUMPTION OF PRION-INFECTED FOODSTUFFS.
CC -!- SIMILARITY: BELONGS TO THE PRION FAMILY.
DR HSSP; P04925; 1AG2. [HSSP ENTRY / SWISS-3DIMAGE / PDB]
DR MIM; 176640; -. [NCBI / EBI]
DR InterPro; IPR000817; -.
DR Pfam; PF00377; prion; 1.
DR PRINTS; PR00341; PRION.
KW Prion; Brain; Glycoprotein; GPI-anchor; Repeat; Signal; Polymorphism; Disease mutation.
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