Lipidoses

Conditions characterized by abnormal lipid deposition due to disturbance in lipid metabolism, such as hereditary diseases involving lysosomal enzymes required for lipid breakdown. They are classified either by the enzyme defect or by the type of lipid involved.

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Networked: 669 relevant articles (17 outcomes, 69 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Atherosclerosis
2.	Weight Gain
3.	Ketosis
4.	Mucopolysaccharidoses
5.	Weight Loss (Weight Reduction)

Experts

1.	Mustonen, Anne-Mari: 5 articles (04/2020 - 03/2009)
2.	Nieminen, Petteri: 5 articles (04/2020 - 03/2009)
3.	Ioannou, Yiannis A: 5 articles (01/2017 - 02/2003)
4.	Oikawa, Shin: 4 articles (01/2019 - 01/2002)
5.	Rouvinen-Watt, Kirsti: 4 articles (09/2010 - 03/2009)
6.	Beaufrère, Hugues: 3 articles (06/2022 - 01/2019)
7.	Favier, Robert P: 3 articles (01/2020 - 11/2015)
8.	Robben, Joris H: 3 articles (01/2020 - 11/2015)
9.	Valtolina, Chiara: 3 articles (01/2020 - 11/2015)
10.	Chen, Fannie W: 3 articles (01/2017 - 09/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lipidoses:

1.	Carnitine (L-Carnitine)FDA LinkGeneric 01/01/2021 - "The aim of this study was to present the case study of patient B.D., a 37-year-old woman diagnosed with muscular lipidosis with L-carnitine deficiency at 6 years old, and describe the speech-language follow-up performed at a hearing care clinic. " 09/01/1990 - "These data suggest that carnitine deficiency does not contribute to the pathogenesis of feline idiopathic hepatic lipidosis." 09/01/1990 - "The mean for the ratio of free/total carnitine in plasma and liver was lower (P less than 0.05) in cats with idiopathic hepatic lipidosis than in control cats. " 09/01/1990 - "The mean concentrations of plasma, liver, and skeletal muscle total carnitine; plasma and skeletal muscle free carnitine; and plasma and liver esterified carnitine were greater (P less than 0.05) in cats with idiopathic hepatic lipidosis than in control cats. " 09/01/1990 - "Concentrations of total, free, and esterified carnitine were determined in plasma, liver, and skeletal muscle from cats with idiopathic hepatic lipidosis and compared with values from healthy cats. "
2.	Fatty Acids (Saturated Fatty Acids)IBA 06/01/2023 - "Furthermore, BST suppressed hepatic lipidosis by decreasing de novo lipogenesis and increasing fatty acid oxidation. " 01/01/2009 - "The content of all these fatty acids increased significantly in the diabetic retina, reflecting lipidosis. " 01/01/2004 - "Results indicate that hepatic lipidosis markedly alters the proportions of the various fatty acids in the liver of dairy cows." 10/01/2003 - "The primary mechanism leading to hepatic lipidosis in cats appears to be decreased fatty acid oxidation. " 01/01/1987 - "Heart organelles appear to incorporate uncommon fatty acids like 22:1 and trans- 18:1. Diets enriched with 22:1 induce myocardial lipidosis. "
3.	Glucose (Dextrose)FDA LinkGeneric 12/01/2021 - "However, at 5 months of age these mice showed markedly increased hepatic lipidosis and became glucose-intolerant. " 01/01/2017 - "The results show that PC-derived exosomes can readily enter C2C12 myotubes, triggering lipidosis and glucose intake inhibition. " 07/01/1997 - "Serum glucose and total carbon dioxide concentrations were significantly lower in cattle with severe lipidosis than in those with mild or moderate lipidosis, and low serum glucose or total carbon dioxide concentrations were rare in cattle without severe lipidosis. " 11/01/2012 - "These findings do not necessarily imply that olanzapine is directly associated with a risk of impairment of weight gain, glucose metabolism disturbances and lipidosis. " 11/01/2012 - "On the other hand, olanzapine has many reports of causing weight gain, glucose metabolism disturbances and lipidosis. "
4.	LipidsIBA 08/01/2017 - "Data from this study suggest that interstitial lipid accumulation may be related to tubular lipidosis (typical of feline kidneys) associated with epithelial degeneration and lysis, and to tubular basement membrane fragmentation. " 04/01/2004 - "This study was designed to monitor lipid profile in the portal and hepatic blood of cows with fasting-induced hepatic lipidosis, and to compare the results with those in the jugular blood. " 07/01/1997 - "The purpose of this study was to characterize the lipid classes in hepatic and adipose tissues from cats with idiopathic hepatic lipidosis (IHL). " 06/01/1988 - "[Importance of fluorescent lipid substrates for the study of hereditary lysosomal lipidoses. " 01/01/1985 - "A comprehensive morphobiochemical study of aortal lipoidosis and a study of the serum lipid content were conducted in children. "
5.	CholesterolIBA 01/01/2013 - "9-fold and 35-fold enrichment in cholesterol, consistent with alveolar lipidosis. " 09/01/2005 - "However, recent data indicate that cholesterol is not the primary culprit in this human lipidosis. " 06/01/2004 - "A low-fat and energy-restricted diet did not reduce either total cholesterol or the corneal lipidosis. " 08/21/2001 - "Niemann-Pick type C (NPC) disease is a cholesterol lipidosis caused by mutations in NPC1 and NPC2 gene loci. " 09/01/1999 - "Niemann-Pick type C (NPC) disease is a severe cell lipidosis characterized by the accumulation of unesterified cholesterol in the endosomal/lysosomal system. "
6.	Insulin (Novolin)FDA Link 08/01/2008 - "Despite hepatic lipidosis, treatment resulted in improved insulin signaling in the liver, muscle, and adipose tissue. " 04/01/2023 - "[The lipidosis of the liver of dairy cows: Part 1 - Role of insulin and the Growth Hormone-IGF-1 axis]." 02/01/2016 - "The insulin in all insulin-depended cells inhibits biological reaction of lipolysis enhancing contributing into development of lipoidosis. " 08/01/2006 - "Decreased insulin response may be an important complication of negative energy balance and hepatic lipidosis." 08/01/2006 - "Decreased insulin response in dairy cows following a four-day fast to induce hepatic lipidosis."
7.	Free RadicalsIBA 01/01/2013 - "If this is also true in ruminants, quercetin could be beneficial in periparturient high-yielding dairy cows by ameliorating the negative effects of free radical formation and reducing the severity of liver lipidosis and ketosis. " 07/01/1998 - "It was shown that inhibition of activity of lipoxygenase during process of development of atherosclerosis led to considerable decrease of area of lipoid plaques and lipoidosis, decreased content of cholesterol in blood and improved free-radical modification of lipoproteins of blood."
8.	Proteins (Proteins, Gene)FDA Link 04/10/2009 - "In conclusion, the regulatory response of identified proteins may help to explain development and consequences of hepatic lipidosis but also offers novel candidates potentially involved in signaling for maintaining energy homeostasis." 02/26/2022 - "A partial least square regression was conducted to identify the proteins associated with liver lipidosis, oxidative stress, and antioxidative capacity. " 04/01/2014 - "However, long-term exposure to low protein provision in mink reduces their growth potential and induces transient hepatic lipidosis and modified body composition." 12/01/2011 - "Deficiency of a Niemann-Pick, type C1-related protein in toxoplasma is associated with multiple lipidoses and increased pathogenicity." 01/01/1983 - "Lysosomes structurally altered by the lipidosis were able to accumulate the protein, although to a smaller extent than normal-appearing lysosomes present in the same cells; the label persisted longer in the altered than in the normal-appearing lysosomes. "
9.	Lipoproteins (Lipoprotein)IBA 04/13/2020 - "Increased delta9-desaturation and secretion of lipoproteins and extracellular vesicles could offer potential mechanisms for further studies to unravel how OA-treated cells alleviate lipidosis." 01/01/2019 - "The aim of this study was to assess serum lipoprotein profiles using rapid single-spin continuous lipoprotein density profiling (CLPDP) in healthy control cats and cats with hepatic lipidosis (HL). " 01/01/2019 - "Altered lipoprotein profiles in cats with hepatic lipidosis." 12/01/1998 - "Characterization of serum lipoprotein profiles of healthy, adult cats and idiopathic feline hepatic lipidosis patients." 02/01/1983 - "These results support the hypothesis that severe hepatic lipidosis in cattle occurs due to impaired hepatic lipoprotein synthesis and secretion."
10.	EnzymesIBA 01/01/2008 - "(1) It is recommended to do the enzyme assays as the first choice in all cases suspected clinically to have lipidoses in order to establish the diagnosis instead of doing invasive procedures as liver and bone marrow biopsies. " 05/01/1995 - "Enzymes utilizing fat in the macrophages were not revealed at early stages of lipoidosis. " 11/01/1994 - "From 1975 to 1993, the activity of various lysosomal enzymes was determined in the leukocytes, cultured skin fibroblasts, or hair roots from 5,594 patients, mainly children, in whom the diagnosis of a lipidosis was suspected. " 03/01/1986 - "The clinical chemical diagnosis of lipidoses can be accomplished by demonstration of the enzyme defect and/or substrate accumulation in body fluids (urine, blood serum), leukocytes, cultured fibroblasts, amniotic fluid cells, or amniotic fluid, respectively. " 01/01/1967 - "[On the problem of the relationship between the activity of lipolytic enzymes and lipoidosis of the aortic wall]."

Therapies and Procedures

1.	Therapeutics 01/01/2017 - "Niemann-Pick type C (NP-C) disease is a fatal genetic lipidosis for which there is no Food and Drug Administration (FDA)-approved therapy. " 01/01/2016 - "Recently the replacement enzymatic therapy availability is substantially modifying the clinical picture and the outcome in some lipidoses such as Anderson-Fabry and Gaucher diseases. " 05/01/2002 - "Most cats (7/9) survived long term postsurgery (mean, 21 months; median, 24 months) without additional medical therapy, while the two cats with concurrent hepatic lipidosis died. " 08/01/2012 - "Therapy is similar to that used in dogs, with added emphasis on early nutritional support to prevent hepatic lipidosis. " 09/01/1984 - "Fatty liver syndrome or hepatic lipidosis (HL) is a condition thought to contribute to an increased incidence of peripartum disease, reduced response to therapy and decreased fertility in dairy cows. "
2.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 12/01/1995 - "Since a successful bone marrow transplantation (BMT) for Hurler's disease reported in 1981, BMT has been established as an effective treatment for lysosomal storage diseases such as mucopolysaccharidosis or lipidosis. " 07/01/1999 - "Niemann-Pick disease type C (a cellular cholesterol lipidosis) treated by bone marrow transplantation."
3.	Glycemic Control 01/01/2022 - "Egg-cellulose, egg-inulin, and whey-inulin diets decreased weight gain, adiposity, and hepatic lipidosis; decreased lipogenic transcripts, improved glycemic control, and upregulated hepatic glucose metabolism transcripts; and decreased plasma insulin and leptin. "
4.	Surgical Portasystemic Shunt (Portosystemic Shunt) 11/01/2014 - "This preliminary study suggests that the presence of hepatic lipidosis and LG has no demonstrable effect on development of acquired shunts or the magnitude of increase in liver volume after attenuation of congenital extrahepatic portosystemic shunts in dogs."
5.	Castration 08/08/2017 - "Since hepatic lipidosis is a frequent disorder in cats, the aim of the current study was to evaluate liver and plasma lipid dimorphism in healthy cats and the effects of gonadectomy on lipid profiling. "