LPG
MCID: LPP002
MIFTS: 46

Lipoprotein Glomerulopathy (LPG) malady

Categories: Genetic diseases, Nephrological diseases, Metabolic diseases, Endocrine diseases, Rare diseases

Aliases & Classifications for Lipoprotein Glomerulopathy

Aliases & Descriptions for Lipoprotein Glomerulopathy:

Name: Lipoprotein Glomerulopathy 54 24 56 66 29 13 69
Lpg 24 56 66

Characteristics:

Orphanet epidemiological data:

56
lipoprotein glomerulopathy
Inheritance: Autosomal dominant; Age of onset: All ages;

Classifications:



External Ids:

OMIM 54 611771
Orphanet 56 ORPHA329481
ICD10 via Orphanet 34 N07.8
MedGen 40 C2673196

Summaries for Lipoprotein Glomerulopathy

OMIM : 54 Lipoprotein glomerulopathy is an uncommon kidney disease characterized by proteinuria, progressive kidney failure, and... (611771) more...

MalaCards based summary : Lipoprotein Glomerulopathy, also known as lpg, is related to zap-70 deficiency and cetp-related hyperalphalipoproteinemia, and has symptoms including renal insufficiency, proteinuria and glomerulopathy. An important gene associated with Lipoprotein Glomerulopathy is APOE (Apolipoprotein E), and among its related pathways/superpathways are Metabolism and Metabolism of water-soluble vitamins and cofactors. The drugs Irbesartan and Sevelamer have been mentioned in the context of this disorder. Affiliated tissues include kidney and endothelial, and related phenotypes are Decreased free cholesterol and Increased LDL uptake

UniProtKB/Swiss-Prot : 66 Lipoprotein glomerulopathy: Uncommon kidney disease characterized by proteinuria, progressive kidney failure, and distinctive lipoprotein thrombi in glomerular capillaries.

Related Diseases for Lipoprotein Glomerulopathy

Diseases related to Lipoprotein Glomerulopathy via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 57)
id Related Disease Score Top Affiliating Genes
1 zap-70 deficiency 10.2 APOB APOE
2 cetp-related hyperalphalipoproteinemia 10.2 APOB APOE
3 joint disorders 10.2 APOB APOE
4 spinocerebellar ataxia 20 10.1 APOB LPA
5 nephrotic syndrome 10.1
6 malignant hyperthermia susceptibility 1 10.1 APOB LPA
7 primary pigmented nodular adrenocortical disease 10.1 APOB APOE
8 anaplastic ganglioglioma 10.1 LCAT LPA
9 gait apraxia 10.1 APOB APOE
10 glaucoma 1, open angle, n 10.1 APOE CLU
11 fetal warfarin syndrome 10.1 APOB LCAT
12 tyrosinemia, type ii 10.0 APOA2 LCAT
13 optic nerve neoplasm 10.0 APOB APOE LPA
14 syringocystadenoma papilliferum 10.0 APOE CLU
15 myelophthisic anemia 10.0 APOB APOE LPA
16 budd-chiari syndrome 10.0 APOB APOE LPA
17 mannosidosis, beta 10.0 APOB APOE LCAT
18 dyserythropoietic anemia, congenital, type ii 10.0 APOE LCAT LPA
19 bleeding disorder, east texas type 10.0 APOA2 APOB APOE
20 pontocerebellar hypoplasia, type 10 10.0 APOE LDLR
21 atherosclerosis 9.9
22 familial progressive cardiac conduction defect 9.9 APOA2 APOE LCAT
23 intra-abdominal lymph node mast cell malignancy 9.9 APOE LPA
24 van maldergem syndrome 2 9.9 APOA2 LPA
25 keratoconus 4 9.9 APOB APOE LDLR
26 severe intellectual disability-progressive postnatal microcephaly- midline stereotypic hand movements syndrome 9.9 APOB APOE LDLR
27 asphyxia neonatorum 9.9 APOE LDLR LPA
28 cervix small cell carcinoma 9.9 APOB APOE LDLR
29 fibrosarcoma of bone 9.9 APOB LDLR LPA
30 chondrocalcinosis with early-onset osteoarthritis 9.8 APOB APOE LCAT LPA
31 albinism, oculocutaneous, type v 9.8 APOA2 APOB APOE LPA
32 von hippel-lindau syndrome 9.8 APOB APOE LCAT LPA
33 skin squamous cell carcinoma 9.7 APOB APOE LDLR LPA
34 autoimmune-related retinopathy and optic neuropathy 9.7 APOB APOE LDLR LPA
35 pericarditis 9.7 APOB APOE LDLR LPA
36 glomerulonephritis 9.7
37 malignant hypertension 9.7
38 psoriasis 9.7
39 fibrillary glomerulonephritis 9.7
40 endotheliitis 9.7
41 dentinogenesis imperfecta type 2 9.7 APOB APOE LCAT LDLR
42 hypercholesterolemia, due to ligand-defective apo b 9.7 APOB APOE LCAT LDLR
43 bird fancier's lung 9.6 APOA2 APOB APOE LCAT LPA
44 neuropathy, hereditary sensory and autonomic, type ia 9.6 APOA2 APOB APOE LCAT LPA
45 maturity-onset diabetes of the young 6 9.6 APOA2 APOB APOE LCAT LPA
46 acute apical periodontitis 9.5 APOB APOE LCAT LDLR LPA
47 lipomatosis 9.5 APOB APOE LCAT LDLR LPA
48 immunodeficiency 18 9.5 APOA2 APOB APOE LDLR LPA
49 pyrimidine metabolic disorder 9.5 APOA2 APOB APOE LDLR LPA
50 myocardial infarction 9.5 APOA2 APOB APOE LDLR LPA

Graphical network of the top 20 diseases related to Lipoprotein Glomerulopathy:



Diseases related to Lipoprotein Glomerulopathy

Symptoms & Phenotypes for Lipoprotein Glomerulopathy

Clinical features from OMIM:

611771

Human phenotypes related to Lipoprotein Glomerulopathy:

32
id Description HPO Frequency HPO Source Accession
1 renal insufficiency 32 HP:0000083
2 proteinuria 32 HP:0000093
3 glomerulopathy 32 HP:0100820
4 mesangial abnormality 32 HP:0001966

GenomeRNAi Phenotypes related to Lipoprotein Glomerulopathy according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased free cholesterol GR00340-A-2 9.26 APOB APOE LDLR LPA
2 Increased LDL uptake GR00340-A-1 8.62 LDLR APOE

MGI Mouse Phenotypes related to Lipoprotein Glomerulopathy:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.35 APOE CLU LCAT LDLR APOB
2 liver/biliary system MP:0005370 8.92 APOB APOE LCAT LDLR

Drugs & Therapeutics for Lipoprotein Glomerulopathy

Drugs for Lipoprotein Glomerulopathy (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 9)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Irbesartan Approved, Investigational 138402-11-6 3749
2
Sevelamer Approved 52757-95-6, 152751-57-0 3085017
3
Uric Acid Experimental, Investigational 69-93-2 1175
4
Angiotensin II 68521-88-0, 11128-99-7 65143 172198
5 Angiotensin II Type 1 Receptor Blockers
6 Angiotensin Receptor Antagonists
7 Angiotensinogen
8 Antihypertensive Agents
9 Chelating Agents

Interventional clinical trials:


id Name Status NCT ID Phase
1 Therapeutic Effect of Immunoadsorption for Patients With Lipoprotein Glomerulopathy Completed NCT00302510
2 A Prospective, Single-center, Randomized, Controlled Study of Sevelamer Carbonate in the Lipid Metabolism and Uric Acid Treatment of Obesity-Related Glomerulopathy Recruiting NCT02644486

Search NIH Clinical Center for Lipoprotein Glomerulopathy

Genetic Tests for Lipoprotein Glomerulopathy

Genetic tests related to Lipoprotein Glomerulopathy:

id Genetic test Affiliating Genes
1 Lipoprotein Glomerulopathy 29 24 APOE

Anatomical Context for Lipoprotein Glomerulopathy

MalaCards organs/tissues related to Lipoprotein Glomerulopathy:

39
Kidney, Endothelial

Publications for Lipoprotein Glomerulopathy

Articles related to Lipoprotein Glomerulopathy:

(show top 50) (show all 84)
id Title Authors Year
1
SPONTANEOUS LIPOPROTEIN GLOMERULOPATHY-LIKE NEPHROPATHY IN A SQUIRREL (SCIURUS VULGARIS). ( 27468047 )
2016
2
Macrophage Infiltration into the Glomeruli in Lipoprotein Glomerulopathy. ( 26955632 )
2015
3
Long-term outcome of kidney transplantation in a patient with coexisting lipoprotein glomerulopathy and fibrillary glomerulonephritis. ( 25852917 )
2014
4
Apolipoprotein e mutation and double filtration plasmapheresis therapy on a new chinese patient with lipoprotein glomerulopathy. ( 25300642 )
2014
5
Macrophage-derived apoESendai suppresses atherosclerosis while causing lipoprotein glomerulopathy in hyperlipidemic mice. ( 25183802 )
2014
6
Apolipoprotein E mutations: a comparison between lipoprotein glomerulopathy and type III hyperlipoproteinemia. ( 24570178 )
2014
7
Lipoprotein glomerulopathy: a case report of a rare disease in a Brazilian child. ( 24676620 )
2014
8
Lipoprotein glomerulopathy may provide a key to unlock the puzzles of renal lipidosis. ( 24487366 )
2014
9
A founder haplotype of APOE-Sendai mutation associated with lipoprotein glomerulopathy. ( 23407349 )
2013
10
Thermodynamic and structural destabilization of apoE3 by hereditary mutations associated with the development of lipoprotein glomerulopathy. ( 23110818 )
2013
11
Lipoprotein glomerulopathy in China. ( 24165683 )
2013
12
Lipoprotein glomerulopathy associated with a mutation in apolipoprotein e. ( 24348079 )
2013
13
A possible structural basis behind the pathogenic role of apolipoprotein E hereditary mutations associated with lipoprotein glomerulopathy. ( 24149834 )
2013
14
Hereditary features, treatment, and prognosis of the lipoprotein glomerulopathy in patients with the APOE Kyoto mutation. ( 24025644 )
2013
15
Topics in lipoprotein glomerulopathy: an overview. ( 24149835 )
2013
16
A case of lipoprotein glomerulopathy with thrombotic microangiopathy due to malignant hypertension. ( 23448537 )
2013
17
[Identification of a mutation in exon 4 of apolipoprotein E gene in a family affected with lipoprotein glomerulopathy]. ( 22487820 )
2012
18
A rare cause of childhood-onset nephrotic syndrome: lipoprotein glomerulopathy. ( 22874113 )
2012
19
Macrophage impairment produced by Fc receptor gamma deficiency plays a principal role in the development of lipoprotein glomerulopathy in concert with apoE abnormalities. ( 22863838 )
2012
20
Scavenger receptor expressions in the kidneys of mice with lipoprotein glomerulopathy. ( 22072188 )
2012
21
A novel mutation ApoE2 Kurashiki (R158P) in a patient with lipoprotein glomerulopathy. ( 21467726 )
2011
22
Significance of a novel apolipoprotein E variant, ApoE Osaka/Kurashiki, in lipoprotein glomerulopathy. ( 21670560 )
2011
23
Lipoprotein glomerulopathy: first report of 2 not consanguineous Italian men from the same town. ( 21534236 )
2011
24
Lipoprotein glomerulopathy. ( 21464714 )
2011
25
A novel apolipoprotein E mutation, ApoE Osaka (Arg158 Pro), in a dyslipidemic patient with lipoprotein glomerulopathy. ( 21325775 )
2011
26
Successful treatment of lipoprotein glomerulopathy in a daughter and a mother using niceritrol. ( 20842518 )
2010
27
A new apolipoprotein E mutation, apoE Las Vegas, in a European-American with lipoprotein glomerulopathy. ( 20624773 )
2010
28
A rare case of lipoprotein glomerulopathy in a white man: an emerging entity in Asia, rare in the white population. ( 20121619 )
2010
29
Atherosclerosis, inflammation and lipoprotein glomerulopathy in kidneys of apoE-/-/LDL-/- double knockout mice. ( 20727187 )
2010
30
Common apolipoprotein E gene mutations contribute to lipoprotein glomerulopathy in China. ( 20090368 )
2010
31
Improvement of nephrotic syndrome by intensive lipid-lowering therapy in a patient with lipoprotein glomerulopathy. ( 19603250 )
2009
32
Lipoprotein glomerulopathy induced by ApoE-Sendai is different from glomerular lesions in aged apoE-deficient mice. ( 19459027 )
2009
33
A rare cause of nephrotic syndrome: lipoprotein glomerulopathy. ( 19197098 )
2009
34
Lipoprotein glomerulopathy treated with LDL-apheresis (Heparin-induced Extracorporeal Lipoprotein Precipitation system): a case report. ( 20062740 )
2009
35
Clinicopathological and genetic characteristics in Chinese patients with lipoprotein glomerulopathy. ( 18264944 )
2008
36
Renal failure with intracapillary thrombi. Lipoprotein glomerulopathy. ( 18414445 )
2008
37
A novel variant apolipoprotein E Okayama in a patient with lipoprotein glomerulopathy. ( 18045818 )
2008
38
A novel apolipoprotein E mutation, ApoE Tsukuba (Arg 114 Cys), in lipoprotein glomerulopathy. ( 17967799 )
2008
39
Identification of apolipoprotein E Guangzhou (arginine 150 proline), a new variant associated with lipoprotein glomerulopathy. ( 18046082 )
2008
40
APOE Kyoto mutation in European Americans with lipoprotein glomerulopathy. ( 18077821 )
2007
41
Lipoprotein glomerulopathy-like disease in a patient with type III hyperlipoproteinemia due to apolipoprotein E2 (Arg158 Cys)/3 heterozygosity. ( 17593519 )
2007
42
A complete genomic analysis of the apolipoprotein E gene in Chinese patients with lipoprotein glomerulopathy. ( 17918142 )
2007
43
Impact of lipoprotein glomerulopathy on the relationship between lipids and renal diseases. ( 16431249 )
2006
44
Lipoprotein glomerulopathy: a new apolipoprotein E mutation with enhanced glomerular binding. ( 16490634 )
2006
45
Plasma level and genetic variation of apolipoprotein E in patients with lipoprotein glomerulopathy. ( 15820086 )
2005
46
[Clinical and pathological analyses of lipoprotein glomerulopathy]. ( 15181857 )
2004
47
Lipoprotein glomerulopathy associated with psoriasis vulgaris: report of 2 cases with apolipoprotein E3/3. ( 12955707 )
2003
48
Disappearance of intraglomerular lipoprotein thrombi and marked improvement of nephrotic syndrome by bezafibrate treatment in a patient with lipoprotein glomerulopathy. ( 12921981 )
2003
49
Resolution of typical lipoprotein glomerulopathy by intensive lipid-lowering therapy. ( 12500244 )
2003
50
Chronic graft-versus-host autoimmune disease in Fc receptor gamma chain-deficient mice results in lipoprotein glomerulopathy. ( 12039982 )
2002

Variations for Lipoprotein Glomerulopathy

UniProtKB/Swiss-Prot genetic disease variations for Lipoprotein Glomerulopathy:

66
id Symbol AA change Variation ID SNP ID
1 APOE p.Arg43Cys VAR_042734 rs121918399
2 APOE p.Arg163Pro VAR_042735 rs121918397

ClinVar genetic disease variations for Lipoprotein Glomerulopathy:

6
id Gene Variation Type Significance SNP ID Assembly Location
1 APOE NM_000041.3(APOE): c.488G> C (p.Arg163Pro) single nucleotide variant Pathogenic rs121918397 GRCh37 Chromosome 19, 45412041: 45412041
2 APOE NM_000041.3(APOE): c.127C> T (p.Arg43Cys) single nucleotide variant Pathogenic rs121918399 GRCh37 Chromosome 19, 45411100: 45411100

Expression for Lipoprotein Glomerulopathy

Search GEO for disease gene expression data for Lipoprotein Glomerulopathy.

Pathways for Lipoprotein Glomerulopathy

Pathways related to Lipoprotein Glomerulopathy according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.54 APOA2 APOB APOE LCAT LDLR LPA
2
Show member pathways
12.17 APOA2 APOB APOE LDLR
3
Show member pathways
11.97 APOA2 APOB APOE LCAT LDLR LPA
4
Show member pathways
11.83 APOB APOE LDLR
5
Show member pathways
11.79 APOA2 APOB APOE LDLR
6 11.09 APOB LPA
7 10.91 APOE LDLR
8
Show member pathways
10.85 APOA2 APOB APOE LCAT LDLR

GO Terms for Lipoprotein Glomerulopathy

Cellular components related to Lipoprotein Glomerulopathy according to GeneCards Suite gene sharing:

(show all 13)
id Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.95 APOA2 APOB APOE CLU LCAT LPA
2 extracellular space GO:0005615 9.88 APOA2 APOB APOE CLU LCAT LDLR
3 endoplasmic reticulum lumen GO:0005788 9.7 APOA2 APOB APOE
4 blood microparticle GO:0072562 9.69 APOA2 APOE CLU
5 early endosome GO:0005769 9.67 APOA2 APOB APOE LDLR
6 clathrin-coated endocytic vesicle membrane GO:0030669 9.5 APOB APOE LDLR
7 endocytic vesicle lumen GO:0071682 9.49 APOB APOE
8 spherical high-density lipoprotein particle GO:0034366 9.46 APOA2 CLU
9 intermediate-density lipoprotein particle GO:0034363 9.43 APOB APOE
10 high-density lipoprotein particle GO:0034364 9.43 APOA2 APOE LCAT
11 very-low-density lipoprotein particle GO:0034361 9.33 APOA2 APOB APOE
12 low-density lipoprotein particle GO:0034362 9.13 APOB APOE LDLR
13 chylomicron GO:0042627 8.8 APOA2 APOB APOE

Biological processes related to Lipoprotein Glomerulopathy according to GeneCards Suite gene sharing:

(show all 36)
id Name GO ID Score Top Affiliating Genes
1 lipid metabolic process GO:0006629 9.95 APOB APOE CLU LCAT LDLR LPA
2 post-translational protein modification GO:0043687 9.85 APOA2 APOB APOE
3 steroid metabolic process GO:0008202 9.85 APOB APOE LCAT LDLR
4 positive regulation of gene expression GO:0010628 9.84 APOB CLU LDLR
5 cellular protein metabolic process GO:0044267 9.83 APOA2 APOB APOE
6 lipid transport GO:0006869 9.83 APOA2 APOB APOE LDLR LPA
7 receptor-mediated endocytosis GO:0006898 9.82 APOB APOE LDLR
8 cholesterol metabolic process GO:0008203 9.77 APOA2 APOB APOE LCAT LDLR
9 retinoid metabolic process GO:0001523 9.75 APOA2 APOB APOE
10 cholesterol efflux GO:0033344 9.73 APOA2 APOB APOE
11 high-density lipoprotein particle remodeling GO:0034375 9.71 APOA2 APOE LCAT
12 cholesterol transport GO:0030301 9.71 APOA2 APOB LCAT LDLR
13 chylomicron assembly GO:0034378 9.7 APOA2 APOB APOE
14 chylomicron remodeling GO:0034371 9.69 APOA2 APOB APOE
15 chylomicron remnant clearance GO:0034382 9.67 APOB APOE LDLR
16 triglyceride metabolic process GO:0006641 9.66 APOA2 APOE
17 phosphatidylcholine biosynthetic process GO:0006656 9.65 APOA2 LCAT
18 triglyceride catabolic process GO:0019433 9.64 APOB APOE
19 low-density lipoprotein particle clearance GO:0034383 9.64 APOB LDLR
20 artery morphogenesis GO:0048844 9.63 APOB APOE
21 high-density lipoprotein particle assembly GO:0034380 9.63 APOA2 APOE
22 lipoprotein catabolic process GO:0042159 9.63 APOB APOE LDLR
23 phospholipid efflux GO:0033700 9.62 APOA2 APOE
24 positive regulation of cholesterol esterification GO:0010873 9.62 APOA2 APOE
25 reverse cholesterol transport GO:0043691 9.62 APOA2 APOE CLU LCAT
26 high-density lipoprotein particle clearance GO:0034384 9.61 APOA2 APOE
27 very-low-density lipoprotein particle remodeling GO:0034372 9.61 APOE LCAT
28 negative regulation of beta-amyloid formation GO:1902430 9.6 APOE CLU
29 very-low-density lipoprotein particle clearance GO:0034447 9.59 APOB APOE
30 protein import GO:0017038 9.58 APOE CLU
31 regulation of beta-amyloid clearance GO:1900221 9.57 APOE CLU
32 low-density lipoprotein particle remodeling GO:0034374 9.56 APOA2 APOB APOE LPA
33 cholesterol homeostasis GO:0042632 9.55 APOA2 APOB APOE LCAT LDLR
34 lipoprotein biosynthetic process GO:0042158 9.26 APOA2 APOB APOE LCAT
35 lipoprotein metabolic process GO:0042157 9.1 APOA2 APOB APOE LCAT LDLR LPA
36 transport GO:0006810 10.06 APOA2 APOB APOE LDLR LPA

Molecular functions related to Lipoprotein Glomerulopathy according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 lipid binding GO:0008289 9.61 APOA2 APOB APOE
2 heparin binding GO:0008201 9.54 APOB APOE LPA
3 phospholipid binding GO:0005543 9.5 APOA2 APOB APOE
4 cholesterol binding GO:0015485 9.46 APOA2 APOE
5 beta-amyloid binding GO:0001540 9.4 APOE CLU
6 lipid transporter activity GO:0005319 9.33 APOA2 APOB APOE
7 phosphatidylcholine-sterol O-acyltransferase activator activity GO:0060228 9.26 APOA2 APOE
8 cholesterol transporter activity GO:0017127 9.13 APOA2 APOB APOE
9 low-density lipoprotein particle receptor binding GO:0050750 8.8 APOB APOE CLU

Sources for Lipoprotein Glomerulopathy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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