Biomaterial Database

Familial Mediterranean fever: analysis of inheritance and current linkage data. 1992

M Shohat, and Y L Danon, and J I Rotter

Department of Pediatrics, Children Hospital, Beilinson Medical Center, Petah Tikva, Israel.

Familial Mediterranean fever (FMF) is a genetic disorder characterized by recurrent attacks of fever and inflammation of serosal surfaces. Unlike many mendelian disorders, the mode of transmission has been subject to some controversy as segregation analysis studies have always demonstrated fewer "observed" than "expected" affected individuals. Despite efforts to map the gene causing FMF, no definite linkage has been yet identified. This review analyses the epidemiologic and genetic characteristics in order to evaluate critically the inheritance of the disease and provide a perspective on the current biochemical and molecular genetic studies whose aim is to locate the gene for this disease.

UI	MeSH Term	Description	Entries
D008040	Genetic Linkage	The co-inheritance of two or more non-allelic GENES due to their being located more or less closely on the same CHROMOSOME.	Genetic Linkage Analysis,Linkage, Genetic,Analyses, Genetic Linkage,Analysis, Genetic Linkage,Genetic Linkage Analyses,Linkage Analyses, Genetic,Linkage Analysis, Genetic
D008877	Middle East	The region of southwest Asia and northeastern Africa usually considered as extending from Libya on the west to Afghanistan on the east. (From Webster's New Geographical Dictionary, 1988)	Gaza Strip,Near East,West Bank,Gaza Strip (Palestine)
D008957	Models, Genetic	Theoretical representations that simulate the behavior or activity of genetic processes or phenomena. They include the use of mathematical equations, computers, and other electronic equipment.	Genetic Models,Genetic Model,Model, Genetic
D010505	Familial Mediterranean Fever	A group of HEREDITARY AUTOINFLAMMATION DISEASES, characterized by recurrent fever, abdominal pain, headache, rash, PLEURISY; and ARTHRITIS. ORCHITIS; benign MENINGITIS; and AMYLOIDOSIS may also occur. Homozygous or compound heterozygous mutations in marenostrin gene encoding PYRIN result in autosomal recessive transmission; simple heterozygous, autosomal dominant form of the disease also exists with mutations in the same gene.	Mediterranean Fever, Familial,Periodic Disease,Periodic Disease, Wolff's,Wolff Periodic Disease,Wolff's Periodic Disease,Benign Paroxysmal Peritonitis,Familial Mediterranean Fever, Autosomal Dominant,Familial Mediterranean Fever, Autosomal Recessive,Familial Paroxysmal Polyserositis,Periodic Peritonitis,Polyserositis, Familial Paroxysmal,Polyserositis, Recurrent,Recurrent Polyserositis,Benign Paroxysmal Peritonitides,Disease, Periodic,Disease, Wolff Periodic,Disease, Wolff's Periodic,Diseases, Periodic,Familial Paroxysmal Polyserositides,Paroxysmal Peritonitides, Benign,Paroxysmal Peritonitis, Benign,Paroxysmal Polyserositides, Familial,Paroxysmal Polyserositis, Familial,Periodic Disease, Wolff,Periodic Disease, Wolffs,Periodic Diseases,Periodic Peritonitides,Peritonitides, Benign Paroxysmal,Peritonitides, Periodic,Peritonitis, Benign Paroxysmal,Peritonitis, Periodic,Polyserositides, Familial Paroxysmal,Polyserositides, Recurrent,Recurrent Polyserositides,Wolffs Periodic Disease
D005787	Gene Frequency	The proportion of one particular in the total of all ALLELES for one genetic locus in a breeding POPULATION.	Allele Frequency,Genetic Equilibrium,Equilibrium, Genetic,Allele Frequencies,Frequencies, Allele,Frequencies, Gene,Frequency, Allele,Frequency, Gene,Gene Frequencies
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000686	Amyloidosis	A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits.	Amyloidoses