DDD
MCID: DNS007
MIFTS: 37

Dense Deposit Disease (DDD) malady

Categories: Rare diseases

Aliases & Classifications for Dense Deposit Disease

Aliases & Descriptions for Dense Deposit Disease:

Name: Dense Deposit Disease 50
Mesangiocapillary Glomerulonephritis Type 2 50 29
Membranoproliferative Glomerulonephritis, Type Ii 69
Membranoproliferative Glomerulonephritis Type Ii 50
Glomerulonephritis Membranoproliferative Type 2 50
Membranoproliferative Glomerulonephritis Type 2 50
Mpgn 2 50
Ddd 50

Classifications:



Summaries for Dense Deposit Disease

NIH Rare Diseases : 50 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 mesangiocapillary glomerulonephritis type 2, is related to complement factor h deficiency and dense deposit disease/membranoproliferative glomerulonephritis type ii. 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 Doxorubicin and Paclitaxel have been mentioned in the context of this disorder. Affiliated tissues include kidney and breast, and related phenotypes are normal and renal/urinary system

Wikipedia : 71 Membranoproliferative glomerulonephritis (\"MPGN\"), also known as mesangiocapillary glomerulonephritis,... more...

Related Diseases for Dense Deposit Disease

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

(show all 48)
id Related Disease Score Top Affiliating Genes
1 complement factor h deficiency 32.9 CFH CFHR5
2 dense deposit disease/membranoproliferative glomerulonephritis type ii 12.2
3 cfhr5-related dense deposit disease / membranoproliferative glomerulonephritis type ii 12.1
4 cfh-related dense deposit disease / membranoproliferative glomerulonephritis type ii 12.1
5 dowling-degos disease 1 11.7
6 c3 glomerulopathy 11.4
7 degenerative disc disease 11.2
8 dowling-degos disease 2 11.2
9 dyschromatosis symmetrica hereditaria 11.1
10 reticulate acropigmentation of kitamura 11.1
11 glomerulonephritis 10.3
12 membranoproliferative glomerulonephritis 10.2
13 glomerulopathy with fibronectin deposits 2 10.1
14 fibrillary glomerulonephritis 10.1
15 immunotactoid glomerulopathy 10.1
16 collagen type iii glomerulopathy 10.1
17 elane-related neutropenia 10.1 CFH CFHR5
18 multiple vertebral anomalies unusual facies 10.1 CFB CFH
19 hand, foot and mouth disease 10.1 CFB CFH
20 endosalpingiosis 10.1 CFB CFH
21 cone-rod dystrophy 17 10.1 CFB CFH
22 placental choriocarcinoma 10.0 CFB CFH
23 immune-complex glomerulonephritis 10.0 C3 CFH
24 adult liposarcoma 10.0 CFB CFH
25 uterine adnexa cancer 9.9 C3 CFH
26 lipodystrophy 9.9
27 familial partial lipodystrophy 9.9
28 rhizomelic chondrodysplasia punctata 9.9 C3 CFB CFH
29 malignant acth producing neoplasm of pituitary gland 9.9 C3 C5
30 bronchiolitis 9.9 C3 CFH CFHR5
31 heart valve disease 9.8 C3 CFH CFHR5
32 thymus adenosquamous carcinoma 9.8 C3 CFB CFH
33 pdgfra-associated chronic eosinophilic leukemia 9.8 C3 CFB CFHR5
34 crescentic glomerulonephritis 9.8
35 endometrioid ovary carcinoma 9.8 C3 C5
36 pneumonia 9.7
37 kidney disease 9.7
38 vasculitis 9.7
39 amyloidosis 9.7
40 acute kidney failure 9.7
41 atherosclerosis 9.7
42 mycoplasmal pneumonia 9.7
43 nephrotic syndrome 9.7
44 retinitis 9.7
45 pemphigus vulgaris 9.7 C3 C5
46 camptodactyly-arthropathy-coxa vara-pericarditis syndrome 9.5 C3 CFB CFH CFHR2 CFHR5
47 chronic progressive external ophthalmoplegia 9.3 C3 C5 CFB CFH CFHR5
48 desmoplastic small round cell tumor 9.2 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:

44
id 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 44)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
2
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
3
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
4
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
5
Mycophenolic acid Approved Phase 2 24280-93-1 446541
6
Mycophenolate mofetil Approved, Investigational Phase 2 128794-94-5 5281078
7
Benzocaine Approved Phase 2 1994-09-7, 94-09-7 2337
8
Bevacizumab Approved, Investigational Phase 2 216974-75-3
9 tannic acid Approved, Nutraceutical Phase 2
10
Doxil Approved June 1999 Phase 2 31703
11 Alkylating Agents Phase 2
12 Mitogens Phase 2
13 Albumin-Bound Paclitaxel Phase 2
14 Topoisomerase Inhibitors Phase 2
15 Hormones Phase 2
16 Angiogenesis Inhibitors Phase 2
17 Angiogenesis Modulating Agents Phase 2
18 Endothelial Growth Factors Phase 2
19 Anti-Bacterial Agents Phase 2,Phase 1
20 Immunoglobulin G Phase 2
21 Immunoglobulins Phase 2
22 Antibodies, Monoclonal Phase 2
23 Immunosuppressive Agents Phase 2,Phase 1
24 Antibiotics, Antitubercular Phase 2,Phase 1
25 Antibodies Phase 2
26 Antimitotic Agents Phase 2
27 Antirheumatic Agents Phase 2
28 Antineoplastic Agents, Alkylating Phase 2
29 Antineoplastic Agents, Phytogenic Phase 2
30 Immunoglobulin A Phase 2
31 Antigen-Antibody Complex Phase 2
32
Aspirin Approved, Vet_approved Phase 1 50-78-2 2244
33
Iron Approved Phase 1 7439-89-6 23925
34
Miconazole Approved, Investigational, Vet_approved Phase 1 22916-47-8 4189
35
Everolimus Approved Phase 1 159351-69-6 6442177
36
Sirolimus Approved, Investigational Phase 1 53123-88-9 5284616 6436030 46835353
37 Vaccines Phase 1
38 Angiotensin-Converting Enzyme Inhibitors Phase 1
39 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 1
40 Anti-Infective Agents Phase 1
41 Antifungal Agents Phase 1
42
Menthol Approved 2216-51-5 16666
43
Iodine 7553-56-2 807
44 cadexomer iodine

Interventional clinical trials:

(show all 11)
id Name Status NCT ID Phase
1 Comparison of Therapeutic Regimens for Scleroderma Interstitial Lung Disease (The Scleroderma Lung Study II) Completed NCT00883129 Phase 2
2 Paclitaxel With or Without Carboplatin and/or Bevacizumab Followed by Doxorubicin and Cyclophosphamide in Treating Patients With Breast Cancer That Can Be Removed by Surgery Completed NCT00861705 Phase 2
3 Safety Study of IgAN, LN, MN, & C3 Glomerulopathy Including Dense Deposit Disease Treated With OMS721 Recruiting NCT02682407 Phase 2
4 A Proof-of-Mechanism Study to Determine the Effect of ACH-0144471 on C3 Levels in Patients With C3G or IC-MPGN Not yet recruiting NCT03124368 Phase 2
5 Eculizumab Therapy for Dense Deposit Disease and C3 Nephropathy Unknown status NCT01221181 Phase 1
6 Clinical Trial of CDX-1135 in Pediatric and Adult Patients With Dense Deposit Disease Terminated NCT01791686 Phase 1
7 Plasmonic Photothermal and Stem Cell Therapy of Atherosclerosis Versus Stenting Terminated NCT01436123 Phase 1
8 Sulodexide Treatment in Patients With Dense Deposit Disease Withdrawn NCT00583427 Phase 1
9 TP10 Use in Patients With C3 Glomerulopathy (C3G) Withdrawn NCT02302755 Phase 1
10 Identification of a New Gene Involved in Hereditary Lipodystrophy Completed NCT02056912
11 Comparison of the Effect of Radial Shock Wave, Ultrasound-guided Needle Puncture, and Combination of Both in the Treatment of Calcific Tendinitis of the Shoulder Completed NCT02677103

Search NIH Clinical Center for Dense Deposit Disease

Genetic Tests for Dense Deposit Disease

Genetic tests related to Dense Deposit Disease:

id Genetic test Affiliating Genes
1 Mesangiocapillary Glomerulonephritis, Type Ii 29

Anatomical Context for Dense Deposit Disease

MalaCards organs/tissues related to Dense Deposit Disease:

39
Kidney, Breast

Publications for Dense Deposit Disease

Articles related to Dense Deposit Disease:

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

id Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.46 C3 C5 CFB CFH
2 blood microparticle GO:0072562 9.13 C3 CFB CFH
3 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:

id Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.56 C3 C5 CFB CFH
2 immune system process GO:0002376 9.54 C3 C5 CFB
3 complement activation, classical pathway GO:0006958 9.46 C3 C5
4 complement activation GO:0006956 9.46 C3 C5 CFB CFH
5 negative regulation of endopeptidase activity GO:0010951 9.43 C3 C5
6 regulation of complement activation GO:0030449 9.43 C3 C5 CFB CFH CFHR2 CFHR5
7 positive regulation of angiogenesis GO:0045766 9.4 C3 C5
8 positive regulation of vascular endothelial growth factor production GO:0010575 9.32 C3 C5
9 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:

id 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
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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