DDD
MCID: DNS007
MIFTS: 46

Dense Deposit Disease (DDD) malady

Rare diseases category
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Summaries for Dense Deposit Disease

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NIH Rare Diseases:42 Dense deposit disease (ddd) is a condition that primarily affects kidney function. signs and symptoms usually start between the ages of 5 and 15 but may also begin in adulthood. the major features of ddd are due to kidney malfunction, and often include proteinuria; hematuria; reduced amounts of urine; low levels of protein in the blood; and swelling in many areas of the body. about half of affected people develop end-stage renal disease (esrd) within 10 years after symptoms start. ddd can have genetic or non-genetic causes. it can be caused by mutations in the c3 and cfh genes; it may develop as a result of both genetic risk factors and environmental triggers; or it can result from the presence of autoantibodies that block the activity of proteins needed for the body's immune response. most cases are sporadic (occurring by chance in people with no history of the disorder in their family). last updated: 2/24/2014

MalaCards based summary: Dense Deposit Disease, also known as mesangiocapillary glomerulonephritis type 2, is related to dense deposit disease/membranoproliferative glomerulonephritis type ii and glomerulonephritis. An important gene associated with Dense Deposit Disease is CFHR5 (complement factor H-related 5), and among its related pathways are Pertussis and Phagocytosis of Microbes. The compounds anaphylatoxin and formate have been mentioned in the context of this disorder. Affiliated tissues include kidney and breast, and related mouse phenotypes are other and normal.

Genetics Home Reference:21 Dense deposit disease is a condition that primarily affects kidney (renal) function. The signs and symptoms usually appear between the ages of 5 and 15, although they can also begin in adulthood.

Wikipedia:65 Membranoproliferative glomerulonephritis (\"MPGN\"), also known as mesangiocapillary glomerulonephritis,... more...

Aliases & Classifications for Dense Deposit Disease

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Dense Deposit Disease, Aliases & Descriptions:

Name: Dense Deposit Disease 42 21
Mesangiocapillary Glomerulonephritis Type 2 42 22 62
Membranoproliferative Glomerulonephritis Type Ii 42 21
Membranoproliferative Glomerulonephritis Type 2 42 62
Mpgnii 21 62
Ddd 42 21
Membranoproliferative Glomerulonephritis, Type Ii 62
 
Glomerulonephritis Membranoproliferative Type 2 42
Glomerulonephritis, Membranoproliferative 62
Type Ii Mpgn 21
Ddd Mpgnii 62
Ddd/mpgnii 21
Mpgn 2 42


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases


Related Diseases for Dense Deposit Disease

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Diseases related to Dense Deposit Disease via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 61)
idRelated DiseaseScoreTop Affiliating Genes
1dense deposit disease/membranoproliferative glomerulonephritis type ii31.1CFH, CFHR5
2glomerulonephritis31.0C3, VTN, CFH
3membranoproliferative glomerulonephritis30.8C3, VTN, CFH
4age related macular degeneration30.3APOE, CFH
5nephrotic syndrome29.8VTN, APOE
6atherosclerosis29.5APOE, VTN, C3
7retinal drusen10.5CFH
8retinitis10.5
9dowling-degos disease 110.4
10dowling-degos disease 210.4
11cfhr5-related dense deposit disease / membranoproliferative glomerulonephritis type ii10.4
12complement factor h deficiency10.4
13dowling-degos disease10.3
14familial partial lipodystrophy10.3
15hepatitis10.3
16niemann-pick disease10.3
17cryoglobulinemia10.3
18hepatitis c10.3
19choroiditis10.3
20lipodystrophy10.3
21adrenocortical carcinoma10.3
22dyschromatosis symmetrica hereditaria 110.3
23atypical hemolytic-uremic syndrome10.3CFH, CFB
24crescentic glomerulonephritis10.3
25cfh-related dense deposit disease / membranoproliferative glomerulonephritis type ii10.3
26hemolytic-uremic syndrome10.2CFH, CFB
27degenerative disc disease10.2
28sick sinus syndrome10.2
29adrenal carcinoma10.2
30amyloidosis10.1
31kidney disease10.1
32pneumonia10.1
33proteinuria10.1
34glomerulopathy with fibronectin deposits 210.1
35immunotactoid glomerulopathy10.1
36mycoplasmal pneumonia10.1
37steroid-resistant nephrotic syndrome10.1
38collagen type iii glomerulopathy10.1
39non-amyloid fibrillary glomerulopathy10.1
40lupus nephritis10.1VTN, CFB
41complement deficiency10.1C3, C5
42myocardial infarction10.0
43inferior myocardial infarction10.0
44phaeochromocytoma10.0
45leydig cell tumor10.0
46malignant leydig cell tumor10.0
47intervertebral disc disease10.0
48superior vena cava syndrome10.0
49multiple sclerosis9.9VTN, APOE
50berger disease9.9C3, VTN, CFH

Graphical network of the top 20 diseases related to Dense Deposit Disease:



Diseases related to dense deposit disease

Symptoms for Dense Deposit Disease

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Drugs & Therapeutics for Dense Deposit Disease

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Drug clinical trials:

Search ClinicalTrials for Dense Deposit Disease

Search NIH Clinical Center for Dense Deposit Disease

Genetic Tests for Dense Deposit Disease

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Genetic tests related to Dense Deposit Disease:

id Genetic test Affiliating Genes
1 Mesangiocapillary Glomerulonephritis, Type Ii22

Anatomical Context for Dense Deposit Disease

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MalaCards organs/tissues related to Dense Deposit Disease:

32
Kidney, Breast

Animal Models for Dense Deposit Disease or affiliated genes

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MGI Mouse Phenotypes related to Dense Deposit Disease:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053958.7CFH, APOE, C3
2MP:00028738.2C5, C3, VTN, CFB, CFH
3MP:00053908.2C5, C3, APOE, CFB
4MP:00053678.1C3, APOE, CFB, CFH
5MP:00053877.5CFH, CFB, APOE, C3, C5
6MP:00053767.3CFH, CFB, APOE, VTN, C3, C5

Publications for Dense Deposit Disease

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Articles related to Dense Deposit Disease:

(show all 38)
idTitleAuthorsYear
1
C3 glomerulopathy: the genetic and clinical findings in dense deposit disease and C3 glomerulonephritis. (24799308)
2014
2
Streptococcal infection as possible trigger for dense deposit disease (C3 glomerulopathy). (24384791)
2014
3
C4 dense-deposit disease. (24552345)
2014
4
Dense deposit disease in an elderly patient: report of a case. (24998957)
2014
5
Heterogeneous histologic and clinical evolution in 3 cases of dense deposit disease with long-term follow-up. (25260719)
2014
6
Eculizumab therapy in a patient with dense-deposit disease associated with partial lipodystropy. (24464478)
2014
7
Blockade of alternative complement pathway in dense deposit disease. (24672732)
2014
8
Eculizumab in dense-deposit disease after renal transplantation. (24908321)
2014
9
Complement factor H-related hybrid protein deregulates complement in dense deposit disease. (24334459)
2014
10
Alternative pathway dysfunction in kidney disease: a case report and review of dense deposit disease and C3 glomerulopathy. (23391537)
2013
11
Childhood-onset dense deposit disease: a rare cause of proteinuria. (24338037)
2013
12
Dense deposit disease and C3 glomerulopathy. (24161036)
2013
13
Dense Deposit Disease: A Rare Cause of Steroid Resistant Nephrotic Syndrome. (23783769)
2013
14
Dense deposit disease: a 29-years electron microscopy experience. (24053741)
2013
15
Eculizumab for the treatment of dense-deposit disease. (22435383)
2012
16
Dense deposit disease in Korean children: a multicenter clinicopathologic study. (23091320)
2012
17
Pathology after eculizumab in dense deposit disease and C3 GN. (22677550)
2012
18
Clinical and pathological features of dense deposit disease in Chinese patients. (22874109)
2012
19
Eculizumab for dense deposit disease and C3 glomerulonephritis. (22403278)
2012
20
Eculizumab in a patient with dense-deposit disease. (22435382)
2012
21
Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. (22456601)
2012
22
Experimental models of membranoproliferative glomerulonephritis, including dense deposit disease. (21252520)
2011
23
A case of dense deposit disease associated with a group A streptococcal infection without the involvement of C3NeF or complement factor H deficiency. (20221776)
2010
24
Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation. (20852386)
2010
25
Anti-factor B autoantibody in dense deposit disease. (20193965)
2010
26
Membranoproliferative glomerulonephritis type II (dense deposit disease) in association with monoclonal gammopathy. (17635761)
2007
27
Dense deposit disease is not a membranoproliferative glomerulonephritis. (17396142)
2007
28
Deletion of Lys224 in regulatory domain 4 of factor H reveals a novel pathomechanism for dense deposit disease (MPGN II). (16612335)
2006
29
Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with membranoproliferative glomerulonephritis type II (dense deposit disease). (16299065)
2006
30
Membranoproliferative glomerulonephritis type II (dense deposit disease): an update. (15800116)
2005
31
Crescentic glomerulonephritis and dense deposit disease in a woman with breast carcinoma on immunosuppressive chemotherapy. (15332237)
2004
32
Crescentic glomerulonephritis in childhood: acute nonproliferative glomerulitis versus dense deposit disease. (12666078)
2003
33
Drusen associated with aging and age-related macular degeneration contain proteins common to extracellular deposits associated with atherosclerosis, elastosis, amyloidosis, and dense deposit disease. (10783137)
2000
34
Dense Deposit Disease/Membranoproliferative Glomerulonephritis Type II (20301598)
1993
35
Porcine membranoproliferative glomerulonephritis with intramembranous dense deposits (porcine dense deposit disease). (8323737)
1993
36
Extensive complement activation in hereditary porcine membranoproliferative glomerulonephritis type II (porcine dense deposit disease). (8238252)
1993
37
Regression of membranoproliferative glomerulonephritis type II (dense deposit disease): observations in six children. (3400634)
1988
38
Mycoplasmal pneumonia associated with mesangiocapillary glomerulonephritis type II (dense deposit disease). (6516912)
1984

Variations for Dense Deposit Disease

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Expression for genes affiliated with Dense Deposit Disease

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Expression patterns in normal tissues for genes affiliated with Dense Deposit Disease

Search GEO for disease gene expression data for Dense Deposit Disease.

Pathways for genes affiliated with Dense Deposit Disease

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Pathways related to Dense Deposit Disease according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.4C5, C3
29.3C3, VTN
3
Show member pathways
Complement Activation, Classical Pathway37
Complement and Coagulation Cascades37
8.6CFH, CFB, C3, C5
48.6CFH, CFB, C3, C5
5
Show member pathways
8.1CFH, CFB, VTN, C3, C5
6
Show member pathways
Immune response Alternative complement pathway60
Immune response Lectin Induced complement pathway60
Immune response Classic complement pathway60
8.1C5, C3, VTN, CFB, CFH

Compounds for genes affiliated with Dense Deposit Disease

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Sources:
44Novoseek, 24HMDB, 11DrugBank, 2BitterDB, 50PharmGKB, 28IUPHAR
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Compounds related to Dense Deposit Disease according to GeneCards/GeneDecks:

(show all 16)
idCompoundScoreTop Affiliating Genes
1anaphylatoxin449.6C3, C5
2formate449.3VTN, APOE
3glycosaminoglycan449.3VTN, CFH
4guanidine44 24 1111.3VTN, APOE
5dermatan sulfate449.2APOE, VTN
6polysaccharide449.2C3, VTN, CFH
7fibrinogen449.2CFH, VTN, C3
8chloroquine44 2 50 28 1113.1VTN, APOE
9sodium dodecylsulfate449.1VTN, APOE
10triacylglycerol449.1C3, APOE
11hyaluronic acid44 2410.1VTN, APOE, CFH
12polyethylene glycol449.0APOE, VTN
13cholesterol44 28 24 1111.4CFH, APOE, VTN, C3
14cysteine448.4C3, VTN, APOE, CFH
15serine448.0CFH, CFB, APOE, VTN, C3
16heparin44 28 24 1110.6C3, CFH, CFHR2, CFHR5, APOE, VTN

GO Terms for genes affiliated with Dense Deposit Disease

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Cellular components related to Dense Deposit Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular vesicular exosomeGO:0700628.1C5, C3, VTN, APOE
2blood microparticleGO:0725627.6CFH, CFB, APOE, VTN, C3
3extracellular spaceGO:0056157.4CFH, APOE, VTN, C3, C5
4extracellular regionGO:0055766.4C5, CFH, CFHR2, CFB, CFHR5, APOE

Biological processes related to Dense Deposit Disease according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1positive regulation vascular endothelial growth factor productionGO:0105759.5C5, C3
2positive regulation of angiogenesisGO:0457669.4C5, C3
3complement activation, classical pathwayGO:0069589.3C5, C3
4negative regulation of blood coagulationGO:0301959.2VTN, APOE
5cellular calcium ion homeostasisGO:0068748.9C5, APOE
6complement activationGO:0069568.8C5, C3, CFB, CFH
7G-protein coupled receptor signaling pathwayGO:0071868.6APOE, C3, C5
8complement activation, alternative pathwayGO:0069578.5C5, C3, CFHR5, CFB, CFH
9regulation of complement activationGO:0304498.3CFH, CFB, VTN, C3, C5
10innate immune responseGO:0450878.2CFH, CFB, VTN, C3, C5

Molecular functions related to Dense Deposit Disease according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1endopeptidase inhibitor activityGO:0048669.4C5, C3
2heparin bindingGO:0082018.5VTN, APOE, CFH

Products for genes affiliated with Dense Deposit Disease

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  • Antibodies
  • Proteins
  • Lysates

Sources for Dense Deposit Disease

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet