MCID: DNS007
MIFTS: 36

Dense Deposit Disease

Categories: Rare diseases

Aliases & Classifications for Dense Deposit Disease

MalaCards integrated aliases for Dense Deposit Disease:

Name: Dense Deposit Disease 49
Membranoproliferative Glomerulonephritis, Type Ii 69
Membranoproliferative Glomerulonephritis Type Ii 49
Glomerulonephritis Membranoproliferative Type 2 49
Membranoproliferative Glomerulonephritis Type 2 49
Mesangiocapillary Glomerulonephritis, Type Ii 28
Mesangiocapillary Glomerulonephritis Type 2 49
Mpgn 2 49
Ddd 49

Classifications:



External Ids:

UMLS 69 C0268743

Summaries for Dense Deposit Disease

NIH Rare Diseases : 49 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: 12/15/2014

MalaCards based summary : Dense Deposit Disease, also known as membranoproliferative glomerulonephritis, type ii, is related to c3 glomerulopathy and glomerulonephritis. An important gene associated with Dense Deposit Disease is CFHR2 (Complement Factor H Related 2), and among its related pathways/superpathways are Creation of C4 and C2 activators and Complement and coagulation cascades. The drugs Immunoglobulin A and Antigen-Antibody Complex have been mentioned in the context of this disorder. Affiliated tissues include kidney, and related phenotypes are normal and renal/urinary system

Related Diseases for Dense Deposit Disease

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

(show all 39)
# Related Disease Score Top Affiliating Genes
1 c3 glomerulopathy 31.7 C3 CFB CFHR5
2 glomerulonephritis 29.9 C3 CFH CFHR5
3 membranoproliferative glomerulonephritis 29.8 C3 CFH CFHR5
4 macular degeneration, age-related, 1 28.5 C3 CFB CFH CFHR5
5 dense deposit disease/membranoproliferative glomerulonephritis type ii 12.3
6 dowling-degos disease 1 11.9
7 complement factor h deficiency 11.7
8 dowling-degos disease 11.2
9 dowling-degos disease 2 11.1
10 glomerulopathy with fibronectin deposits 2 10.2
11 fibrillary glomerulonephritis 10.2
12 immunotactoid glomerulopathy 10.2
13 collagen type iii glomerulopathy 10.2
14 non-immunoglobulin-mediated membranoproliferative glomerulonephritis 10.1
15 blood group, i system 10.1
16 multifocal choroiditis 10.0 CFB CFH
17 choroiditis 10.0 CFB CFH
18 proliferative glomerulonephritis 10.0 C3 CFH
19 kuhnt-junius degeneration 10.0 CFB CFH
20 lyme disease 9.9 C3 CFH
21 hemolytic uremic syndrome, atypical 1 9.9 CFH CFHR5
22 crescentic glomerulonephritis 9.9
23 degeneration of macula and posterior pole 9.9 CFB CFH
24 complement deficiency 9.8 C3 C5
25 aging 9.7
26 acute kidney failure 9.7
27 nephrotic syndrome 9.7
28 retinitis 9.7
29 pneumonia 9.7
30 kidney disease 9.7
31 vasculitis 9.7
32 amyloidosis 9.7
33 mycoplasmal pneumonia 9.7
34 retinal disease 9.7 CFB CFH
35 retinal drusen 9.7 C3 CFB CFH
36 hemoglobinuria 9.6 C3 C5
37 enterocolitis 9.2 C3 CFB CFH CFHR5
38 systemic lupus erythematosus 9.0 C3 C5 CFB
39 hemolytic-uremic syndrome 8.3 C3 C5 CFB CFH CFHR2 CFHR5

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



Diseases related to Dense Deposit Disease

Symptoms & Phenotypes for Dense Deposit Disease

MGI Mouse Phenotypes related to Dense Deposit Disease:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 normal MP:0002873 9.26 C3 C5 CFB CFH
2 renal/urinary system MP:0005367 8.92 C3 C5 CFB CFH

Drugs & Therapeutics for Dense Deposit Disease

Drugs for Dense Deposit Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 6)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunoglobulin A Phase 2
2 Antigen-Antibody Complex Phase 2
3
Aspirin Approved, Vet_approved Phase 1 50-78-2 2244
4 Vaccines Phase 1
5 Angiotensin-Converting Enzyme Inhibitors Phase 1
6 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Safety Study of IgAN, LN, MN, & C3 Glomerulopathy Including Dense Deposit Disease Treated With OMS721 Recruiting NCT02682407 Phase 2
2 A Proof-of-Mechanism Study to Determine the Effect of ACH-0144471 on C3 Levels in Patients With C3G or IC-MPGN Recruiting NCT03124368 Phase 2 ACH-0144471
3 Controlled Trial Evaluating Avacopan in C3 Glomerulopathy Recruiting NCT03301467 Phase 2 Avacopan;Avacopan Matching Placebo
4 Phase II Study Assessing Safety and Efficacy of APL-2 in Glomerulopathies Recruiting NCT03453619 Phase 2 APL-2
5 A Proof of Concept Study for 6 Month Treatment in Patients With C3 Glomerulopathy (C3G) Not yet recruiting NCT03369236 Phase 2 ACH-0144471;Placebo
6 Eculizumab Therapy for Dense Deposit Disease and C3 Nephropathy Unknown status NCT01221181 Phase 1 Eculizumab
7 Clinical Trial of CDX-1135 in Pediatric and Adult Patients With Dense Deposit Disease Terminated NCT01791686 Phase 1 CDX-1135
8 Sulodexide Treatment in Patients With Dense Deposit Disease Withdrawn NCT00583427 Phase 1 Sulodexide
9 TP10 Use in Patients With C3 Glomerulopathy (C3G) Withdrawn NCT02302755 Phase 1 TP10

Search NIH Clinical Center for Dense Deposit Disease

Genetic Tests for Dense Deposit Disease

Genetic tests related to Dense Deposit Disease:

# Genetic test Affiliating Genes
1 Mesangiocapillary Glomerulonephritis, Type Ii 28

Anatomical Context for Dense Deposit Disease

MalaCards organs/tissues related to Dense Deposit Disease:

38
Kidney

Publications for Dense Deposit Disease

Articles related to Dense Deposit Disease:

(show all 48)
# Title Authors Year
1
C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy. ( 29310824 )
2018
2
Treatment with bortezomib in dense deposit disease associated with monoclonal gammopathy of undetermined significance. ( 29363742 )
2018
3
Dense deposit disease associated with multiple myelomaa8c. ( 29208205 )
2017
4
Dense deposit disease in a child with febrile sore throat. ( 28748900 )
2017
5
Proliferative C4 Dense Deposit Disease, Acute ThromboticA Microangiopathy, a Monoclonal Gammopathy, andA Acute Kidney Failure. ( 26704376 )
2016
6
Discontinuation of dialysis with eculizumab therapy in a pediatric patient with dense deposit disease. ( 26759144 )
2016
7
Overlap of ultrastructural findings in C3 glomerulonephritis and dense deposit disease. ( 26649668 )
2015
8
AJKD Atlas of Renal Pathology: Dense Deposit Disease. ( 26300205 )
2015
9
Dense Deposit Disease Mimicking a Renal Small Vessel Vasculitis. ( 26361799 )
2015
10
Eculizumab in Pediatric Dense Deposit Disease. ( 26316621 )
2015
11
Eculizumab in dense-deposit disease after renal transplantation. ( 24908321 )
2014
12
Dense deposit disease in an elderly patient: report of a case. ( 24998957 )
2014
13
Heterogeneous histologic and clinical evolution in 3 cases of dense deposit disease with long-term follow-up. ( 25260719 )
2014
14
Blockade of alternative complement pathway in dense deposit disease. ( 24672732 )
2014
15
C3 glomerulopathy: the genetic and clinical findings in dense deposit disease and C3 glomerulonephritis. ( 24799308 )
2014
16
Eculizumab therapy in a patient with dense-deposit disease associated with partial lipodystropy. ( 24464478 )
2014
17
C4 dense-deposit disease. ( 24552345 )
2014
18
Streptococcal infection as possible trigger for dense deposit disease (C3 glomerulopathy). ( 24384791 )
2014
19
Complement factor H-related hybrid protein deregulates complement in dense deposit disease. ( 24334459 )
2014
20
Dense deposit disease and C3 glomerulopathy. ( 24161036 )
2013
21
Dense deposit disease: a 29-years electron microscopy experience. ( 24053741 )
2013
22
Dense Deposit Disease: A Rare Cause of Steroid Resistant Nephrotic Syndrome. ( 23783769 )
2013
23
Childhood-onset dense deposit disease: a rare cause of proteinuria. ( 24338037 )
2013
24
Alternative pathway dysfunction in kidney disease: a case report and review of dense deposit disease and C3 glomerulopathy. ( 23391537 )
2013
25
Dense deposit disease in Korean children: a multicenter clinicopathologic study. ( 23091320 )
2012
26
Pathology after eculizumab in dense deposit disease and C3 GN. ( 22677550 )
2012
27
Eculizumab in a patient with dense-deposit disease. ( 22435382 )
2012
28
Eculizumab for dense deposit disease and C3 glomerulonephritis. ( 22403278 )
2012
29
Clinical and pathological features of dense deposit disease in Chinese patients. ( 22874109 )
2012
30
Eculizumab for the treatment of dense-deposit disease. ( 22435383 )
2012
31
Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. ( 22456601 )
2012
32
Experimental models of membranoproliferative glomerulonephritis, including dense deposit disease. ( 21252520 )
2011
33
Anti-factor B autoantibody in dense deposit disease. ( 20193965 )
2010
34
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
35
Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation. ( 20852386 )
2010
36
Membranoproliferative glomerulonephritis type II (dense deposit disease) in association with monoclonal gammopathy. ( 17635761 )
2007
37
Dense deposit disease is not a membranoproliferative glomerulonephritis. ( 17396142 )
2007
38
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
39
Deletion of Lys224 in regulatory domain 4 of factor H reveals a novel pathomechanism for dense deposit disease (MPGN II). ( 16612335 )
2006
40
Membranoproliferative glomerulonephritis type II (dense deposit disease): an update. ( 15800116 )
2005
41
Crescentic glomerulonephritis and dense deposit disease in a woman with breast carcinoma on immunosuppressive chemotherapy. ( 15332237 )
2004
42
Crescentic glomerulonephritis in childhood: acute nonproliferative glomerulitis versus dense deposit disease. ( 12666078 )
2003
43
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
44
Dense Deposit Disease/Membranoproliferative Glomerulonephritis Type II ( 20301598 )
1993
45
Extensive complement activation in hereditary porcine membranoproliferative glomerulonephritis type II (porcine dense deposit disease). ( 8238252 )
1993
46
Porcine membranoproliferative glomerulonephritis with intramembranous dense deposits (porcine dense deposit disease). ( 8323737 )
1993
47
Regression of membranoproliferative glomerulonephritis type II (dense deposit disease): observations in six children. ( 3400634 )
1988
48
Mycoplasmal pneumonia associated with mesangiocapillary glomerulonephritis type II (dense deposit disease). ( 6516912 )
1984

Variations for Dense Deposit Disease

Expression for Dense Deposit Disease

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

Pathways for Dense Deposit Disease

GO Terms for Dense Deposit Disease

Cellular components related to Dense Deposit Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.56 C3 C5 CFB CFH
2 protein complex GO:0043234 9.33 C3 CFHR2 CFHR5
3 blood microparticle GO:0072562 9.13 C3 CFB CFH
4 extracellular region GO:0005576 9.1 C3 C5 CFB CFH CFHR2 CFHR5

Biological processes related to Dense Deposit Disease according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.62 C3 C5 CFB CFH
2 immune system process GO:0002376 9.61 C3 C5 CFB
3 complement activation, classical pathway GO:0006958 9.49 C3 C5
4 positive regulation of angiogenesis GO:0045766 9.48 C3 C5
5 negative regulation of endopeptidase activity GO:0010951 9.46 C3 C5
6 complement activation GO:0006956 9.46 C3 C5 CFB CFH
7 regulation of complement activation GO:0030449 9.43 C3 C5 CFB CFH CFHR2 CFHR5
8 negative regulation of protein binding GO:0032091 9.4 CFHR2 CFHR5
9 positive regulation of vascular endothelial growth factor production GO:0010575 9.37 C3 C5
10 positive regulation of cytolysis GO:0045919 9.26 CFHR2 CFHR5
11 complement activation, alternative pathway GO:0006957 9.02 C3 C5 CFB CFH CFHR5

Molecular functions related to Dense Deposit Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endopeptidase inhibitor activity GO:0004866 8.62 C3 C5

Sources for Dense Deposit Disease

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