Familial Partial Lipodystrophy (Dunnigan Syndrome)

78  relevant articles (3 outcomes, 3 trials/studies) found for this Disease

Dunnigan Syndrome; Lipodystrophy, Familial Partial; Familial Partial Lipodystrophy, Kobberling Type; Familial Partial Lipodystrophy, Type 1; Familial Partial Lipodystrophy, Type 2; Familial Partial Lipodystrophy, Type 3; Koberling-Dunnigan Syndrome; Lipodystrophy, Familial Partial, Dunnigan Type; Lipodystrophy, Familial Partial, Type 1; Lipodystrophy, Familial Partial, Type 2; Lipodystrophy, Familial Partial, Type 3; Familial Partial Lipodystrophies; Koberling Dunnigan Syndrome; Lipodystrophies, Familial Partial; Partial Lipodystrophies, Familial; Partial Lipodystrophy, Familial; Syndrome, Dunnigan

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Disease Context: Research Results

Related Diseases

1.	Dilated Cardiomyopathy (Cardiomyopathy, Congestive)
2.	Diabetes Mellitus
3.	Werner Syndrome (Werner's Syndrome)
4.	Polycystic Ovary Syndrome (Syndrome, Stein-Leventhal)
5.	Heart Diseases (Heart Disease)

Experts

1.	Hegele, R A: 3 articles (10/2006 - 01/2000)
2.	Cao, H: 3 articles (10/2006 - 01/2000)
3.	Guerci, B: 2 articles (11/2007 - 11/2000)
4.	Vigouroux, C: 2 articles (11/2007 - 11/2000)
5.	Lascols, O: 2 articles (11/2007 - 11/2000)
6.	Wehnert, Manfred: 2 articles (06/2007 - 11/2003)
7.	Jeninga, Ellen H: 2 articles (05/2007 - 05/2007)
8.	Kalkhoven, Eric: 2 articles (05/2007 - 05/2007)
9.	Genschel, J: 2 articles (11/2005 - 04/2000)
10.	Garg, A: 2 articles (01/2001 - 04/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Familial Partial Lipodystrophy:

1.	LeptinIBA 04/01/2007 - "Long-term efficacy of leptin replacement in patients with Dunnigan-type familial partial lipodystrophy." 05/01/2002 - "Therefore, we compared fasting serum concentrations of adiponectin and leptin, in 18 patients with congenital generalized lipodystrophy (CGL), 11 with acquired generalized lipodystrophy (AGL), 46 with familial partial lipodystrophy-Dunnigan variety (FPLD) and 18 with acquired partial lipodystrophy (APL) and studied their relationship to metabolic parameters" Order ALL the reference details at left...
2.	rosiglitazone (Avandia)FDA Link 11/01/2005 - "Long-term treatment experience in a subject with Dunnigan-type familial partial lipodystrophy: efficacy of rosiglitazone." 10/01/2003 - "Response to treatment with rosiglitazone in familial partial lipodystrophy due to a mutation in the LMNA gene." Order ALL the reference details at left...
3.	pioglitazone (Actos)FDA Link 11/01/2007 - "Efficacy of pioglitazone in familial partial lipodystrophy of the Dunnigan type: a case report." Order ALL the reference details at left...
4.	Lamin Type A (Lamin A)IBA 11/01/2007 - "The detection of a heterozygous missense mutation in LAMIN A/C gene at position 482 confirmed the diagnosis of Familial Partial Lipodystrophy (FPLD2)" 03/01/2007 - "Mutations in the LMNA gene, encoding the nuclear envelope protein lamin A/C, are responsible for a number of distinct disease entities including Dunnigan-type familial partial lipodystrophy" 07/01/2006 - "CONTEXT: To date, all cases of familial partial lipodystrophy type 2 (FPLD2; Mendelian Inheritance in Man 151660) result from missense mutations in LMNA, which encodes nuclear lamin A/C (Mendelian Inheritance in Man 150330)" 02/15/2006 - "Nuclear lamin A inhibits adipocyte differentiation: implications for Dunnigan-type familial partial lipodystrophy." 07/01/2003 - "Twenty-six subjects (9.7%) were positive for a beta-cell antibody, one subject had familial partial lipodystrophy and the lamin A/C mutation R482W, and two subjects had the mitochondrial mutation A3243G" Order ALL the reference details at left...
5.	Proteins (Proteins, Gene)IBA 05/01/2003 - "OBJECTIVES: Familial partial lipodystrophy, Dunnigan variety (FPLD), is an autosomal dominant disorder due to missense mutations in the lamin A/C (LMNA) gene encoding nuclear lamina proteins" 03/01/2007 - "Mutations in LMNA encoding lamin A and C proteins cause monogenic syndromes characterized by muscular dystrophy and familial partial lipodystrophy" 06/10/2007 - "Mutations in genes encoding the intermediate filament nuclear lamins and associated proteins cause a wide spectrum of diseases sometimes called "laminopathies." Diseases caused by mutations in LMNA encoding A-type lamins include autosomal dominant Emery-Dreifuss muscular dystrophy and related myopathies, Dunnigan-type familial partial lipodystrophy, Charcot-Marie-Tooth disease type 2B1 and developmental and accelerated aging disorders" 06/01/2000 - "It is still not at all clear how defects in these nuclear membrane proteins are related to the phenotype, even less clear that LMNA mutations can also be associated with familial dilated cardiomyopathy with no weakness, and even familial partial lipodystrophy with diabetes mellitus and coronary heart disease! What began as clinical studies in a relatively rare form of dystrophy has progressed to detailed research into the functions of nuclear membrane proteins particularly in regard to various forms of heart disease." 08/01/2002 - "The LMNA gene encoding two nuclear envelope proteins (lamins A and C [lamin A/C]) maps to chromosome 1q21 and has been associated with five distinct pathologies, including Dunnigan-type familial partial lipodystrophy, a condition that is characterized by subcutaneous fat loss and is invariably associated with insulin resistance and diabetes" Order ALL the reference details at left...
6.	Naphazoline (Clear, All)FDA LinkGeneric 06/01/2000 - "It is still not at all clear how defects in these nuclear membrane proteins are related to the phenotype, even less clear that LMNA mutations can also be associated with familial dilated cardiomyopathy with no weakness, and even familial partial lipodystrophy with diabetes mellitus and coronary heart disease! What began as clinical studies in a relatively rare form of dystrophy has progressed to detailed research into the functions of nuclear membrane proteins particularly in regard to various forms of heart disease." Order ALL the reference details at left...
7.	LaminsIBA 11/01/2005 - "Dunnigan-type familial partial lipodystrophy (FPLD) is caused by mutations in LMNA, the gene that encodes nuclear lamins A and C" 11/15/2003 - "Familial partial lipodystrophy is an autosomal dominant disease caused by mutations of the LMNA gene encoding alternatively spliced lamins A and C" 06/01/2001 - "We discovered that rare mutations in LMNA, which encodes lamins A and C, underlie autosomal dominant Dunnigan-type familial partial lipodystrophy" 01/01/2001 - "Familial partial lipodystrophy (FPLD) has been shown to be due to mutations in the LMNA gene encoding nuclear lamins A and C, indicating that defective structure of the nuclear envelope can produce this unique phenotype" 09/01/2000 - "Mutations in LMNA, which encodes lamins A and C, have been found in patients with autosomal dominant Dunnigan-type familial partial lipodystrophy (FPLD)" Order ALL the reference details at left...
8.	PPAR gammaIBA 04/01/2008 - "RECENT FINDINGS: Familial partial lipodystrophy is a rare but characteristic phenotype associated with carriers of peroxisome proliferator-activated receptor-gamma missense mutations" 05/01/2007 - "Impaired peroxisome proliferator-activated receptor gamma function through mutation of a conserved salt bridge (R425C) in familial partial lipodystrophy." 10/01/2006 - "A frameshift mutation in peroxisome-proliferator-activated receptor-gamma in familial partial lipodystrophy subtype 3 (FPLD3; MIM 604367)." 01/01/2002 - "A novel heterozygous mutation in peroxisome proliferator-activated receptor-gamma gene in a patient with familial partial lipodystrophy." 01/01/2005 - "Genetic forms of partial lipodystrophy are currently recognized as two syndromes with subcutaneous lipoatrophy but preserved or increased fat at the level of face and neck (Dunnigan syndrome or FPLD due to LMNA mutations) and/or abdomen (PPARgamma-linked forms) and are both transmitted as dominant diseases" Order ALL the reference details at left...
9.	Insulin (Novolin)FDA Link 04/01/1986 - "Partial lipoatrophy with insulin resistant diabetes and hyperlipidaemia (Dunnigan syndrome)." 01/15/1999 - "One subtype, familial partial lipodystrophy Dunnigan (FPLD), is a rare autosomal dominant trait that results in an gradual loss of subcutaneous fat in the lower trunk and limbs, Type V hyperlipoproteinemia, hypertriglyceridemia, and insulin-resistant diabetes" 08/01/2002 - "Rare mutations in LMNA were shown to cause familial partial lipodystrophy, a syndrome characterized by regional loss of adipose tissue, glucose intolerance, and dyslipidemia, making LMNA a candidate gene for insulin-resistant diabetes" 04/01/2000 - "Mutations of the lamin A/C gene causing familial partial lipodystrophy: significance for the development of hyperlipidemia and insulin resistant diabetes mellitus]" Order ALL the reference details at left...
10.	Codon (Codons)IBA 05/08/2001 - "Cardiovascular end points were thus evaluated in subjects with Dunnigan-type familial partial lipodystrophy (FPLD) due to mutations at LMNA codon 482" 12/01/2007 - "OBJECTIVE, DESIGN, SETTING, AND PATIENTS: Sequencing of the LMNA coding regions in 277 unrelated adults investigated for lipodystrophy and/or insulin resistance revealed 17 patients with substitutions at codon 482 observed in typical Dunnigan's familial partial lipodystrophy and 10 patients with other mutations" 09/01/2001 - "Gene analysis of the proband revealed Tyr481His mutation in the exon 8 of lamin A/C (LMNA) gene which is adjacent to the codon mutated in reported cases of familial partial lipodystrophy" Order ALL the reference details at left...

Therapies and Procedures

1.	Highly Active Antiretroviral Therapy (HAART) 05/30/2003 - "Familial partial lipodystrophy (FPL) and lipodystrophy observed in HIV-1 infected patients receiving highly active antiretroviral therapy (HAART) share multiple clinical and metabolic features" Order ALL the reference details at left...
2.	Gastric Bypass (Roux-en-Y Gastric Bypass) 06/01/2006 - "Effectiveness of gastric bypass surgery in a patient with familial partial lipodystrophy." Order ALL the reference details at left...