DDD
MCID: C3G002
MIFTS: 37

C3 Glomerulopathy (DDD)

Aliases & Classifications for C3 Glomerulopathy

MalaCards integrated aliases for C3 Glomerulopathy:

Name: C3 Glomerulopathy 24 25
C3 Glomerulonephritis 25 29 73
C3g 24 25
Membranoproliferative Glomerulonephritis, Type Ii 73
Membranoproliferative Glomerulonephritis Type Ii 25
Glomerulonephritis with Dominant C3 24
Dense Deposit Disease 25
Ddd/mpgnii 25
Ddd 25

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Summaries for C3 Glomerulopathy

Genetics Home Reference : 25 C3 glomerulopathy is a group of related conditions that cause the kidneys to malfunction. The major features of C3 glomerulopathy include high levels of protein in the urine (proteinuria), blood in the urine (hematuria), reduced amounts of urine, low levels of protein in the blood, and swelling in many areas of the body. Affected individuals may have particularly low levels of a protein called complement component 3 (or C3) in the blood.

MalaCards based summary : C3 Glomerulopathy, also known as c3 glomerulonephritis, is related to glomerulonephritis and dense deposit disease. An important gene associated with C3 Glomerulopathy is C3 (Complement C3), and among its related pathways/superpathways are Creation of C4 and C2 activators and Complement and coagulation cascades. The drugs Daratumumab and Complement System Proteins have been mentioned in the context of this disorder. Affiliated tissues include kidney, skin and testes.

GeneReviews: NBK1425

Related Diseases for C3 Glomerulopathy

Diseases related to C3 Glomerulopathy via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 81)
# Related Disease Score Top Affiliating Genes
1 glomerulonephritis 30.5 C3 CFHR5 CFP
2 dense deposit disease 30.2 C3 CFB CFHR5
3 membranoproliferative glomerulonephritis 29.9 C3 CFB CFHR5 CFP
4 hemolytic-uremic syndrome 29.7 C3 CFB CFHR1 CFHR3 CFHR5
5 hemolytic uremic syndrome, atypical 1 29.4 C3 CFB CFHR1 CFHR3 CFHR5 CFP
6 macular degeneration, age-related, 1 28.8 C3 CFB CFHR1 CFHR3 CFHR5
7 dowling-degos disease 1 12.0
8 non-immunoglobulin-mediated membranoproliferative glomerulonephritis 11.8
9 degenerative disc disease 11.5
10 dowling-degos disease 11.4
11 dowling-degos disease 2 11.3
12 acne inversa, familial, 2, with or without dowling-degos disease 11.0
13 atrioventricular block 10.4
14 glomerulopathy with fibronectin deposits 2 10.4
15 fibrillary glomerulonephritis 10.4
16 immunotactoid glomerulopathy 10.4
17 collagen type iii glomerulopathy 10.4
18 adrenocortical carcinoma, hereditary 10.3
19 sick sinus syndrome 10.3
20 adrenal cortical adenocarcinoma 10.3
21 neuroblastoma 10.2
22 atrial standstill 1 10.2
23 leukemia 10.1
24 myeloid leukemia 10.1
25 adrenal carcinoma 10.1
26 lipodystrophy, partial, acquired 10.1
27 complement factor h deficiency 10.1
28 kidney disease 10.1
29 malignant atrophic papulosis 10.0
30 atrial fibrillation 10.0
31 proliferative glomerulonephritis 10.0
32 crescentic glomerulonephritis 10.0
33 monoclonal gammopathy of uncertain significance 10.0
34 hepatitis 10.0
35 niemann-pick disease 10.0
36 hepatitis c 10.0
37 cryoglobulinemia 10.0
38 complement component 3 deficiency 10.0 C3 CFP
39 endocarditis 10.0
40 cervical squamous cell carcinoma 10.0
41 lymphoma 10.0
42 generalized epilepsy with febrile seizures plus 10.0
43 squamous cell carcinoma 10.0
44 complement factor i deficiency 10.0 C3 CFP
45 neisseria meningitidis infection 10.0 C3 CFP
46 cystic fibrosis 10.0
47 myeloma, multiple 10.0
48 retinal disease 10.0
49 retinal drusen 10.0 C3 CFB
50 complement deficiency 9.9 C3 CFP

Graphical network of the top 20 diseases related to C3 Glomerulopathy:



Diseases related to C3 Glomerulopathy

Symptoms & Phenotypes for C3 Glomerulopathy

Drugs & Therapeutics for C3 Glomerulopathy

Drugs for C3 Glomerulopathy (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Daratumumab Approved Phase 2 945721-28-8
2 Complement System Proteins Phase 2,Phase 1
3 Immunologic Factors Phase 2,Phase 1,Not Applicable
4 Complement C3 Phase 2,Phase 1
5 Immunoglobulin A Phase 2
6 Antibodies Phase 2,Not Applicable
7 Immunoglobulins Phase 2,Not Applicable
8 Antigen-Antibody Complex Phase 2,Not Applicable
9 Antibodies, Monoclonal Phase 2
10 Complement Inactivating Agents Phase 1
11 Immunosuppressive Agents Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Controlled Trial Evaluating Avacopan in C3 Glomerulopathy Recruiting NCT03301467 Phase 2 Avacopan;Avacopan Matching Placebo
2 Safety Study of IgAN, LN, MN, & C3 Glomerulopathy Including Dense Deposit Disease Treated With OMS721 Recruiting NCT02682407 Phase 2
3 A Proof of Concept Study for 6 Month Treatment in Patients With C3 Glomerulopathy (C3G) Recruiting NCT03369236 Phase 2 ACH-0144471;Placebo
4 A Proof of Concept Study for a 12 Month Treatment in Patients With C3 Glomerulopathy (C3G) or Immune-Complex Membranoproliferative Glomerulonephritis (IC-MPGN) Recruiting NCT03459443 Phase 2 ACH-0144471
5 Daratumumab in Treatment of PGNMID and C3 GN Recruiting NCT03095118 Phase 2 Daratumumab
6 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
7 Phase II Study Assessing Safety and Efficacy of APL-2 in Glomerulopathies Recruiting NCT03453619 Phase 2 APL-2
8 First-In-Human Clinical Study of the C3 Complement Inhibitor AMY- 101 in Healthy Male Volunteers Completed NCT03316521 Phase 1 AMY-101
9 TP10 Use in Patients With C3 Glomerulopathy (C3G) Withdrawn NCT02302755 Phase 1 TP10
10 Non-contrast Enhanced MRI in Patients With C3 Glomerulopathy (C3G) or Immune-complex Membranoproliferative Glomerulonephritis (IC-MPGN) Enrolled in the ACH471-205 Study Not yet recruiting NCT03723512 Not Applicable

Search NIH Clinical Center for C3 Glomerulopathy

Genetic Tests for C3 Glomerulopathy

Genetic tests related to C3 Glomerulopathy:

# Genetic test Affiliating Genes
1 C3 Glomerulonephritis 29

Anatomical Context for C3 Glomerulopathy

MalaCards organs/tissues related to C3 Glomerulopathy:

41
Kidney, Skin, Testes, B Cells, Lung, Myeloid

Publications for C3 Glomerulopathy

Articles related to C3 Glomerulopathy:

(show top 50) (show all 155)
# Title Authors Year
1
Eculizumab as salvage therapy for recurrent monoclonal gammopathy-induced C3 glomerulopathy in a kidney allograft. ( 29724174 )
2018
2
Mycophenolate Mofetil in Combination with Steroids for Treatment of C3 Glomerulopathy: A Case Series. ( 29326307 )
2018
3
Evolving complexity of complement-related diseases: C3 glomerulopathy and atypical haemolytic uremic syndrome. ( 29517501 )
2018
4
Which should be the correct treatment for monoclonal gammopathy of renal significance with complement alternative pathway dysregulation (C3 glomerulopathy and atypical hemolytic uremic syndrome): clone-directed or anticomplement therapy? ( 29808447 )
2018
5
C3 glomerulopathy associated with monoclonal IgA is a distinct subtype. ( 29729982 )
2018
6
Patterns of Clinical Response to Eculizumab in Patients With C3 Glomerulopathy. ( 29429752 )
2018
7
Genetic analysis of the complement pathway in C3 glomerulopathy. ( 29566171 )
2018
8
C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy. ( 29310824 )
2018
9
C3 glomerulopathy in cystic fibrosis: a case report. ( 29592796 )
2018
10
Autoimmune abnormalities of the alternative complement pathway in membranoproliferative glomerulonephritis and C3 glomerulopathy. ( 29948306 )
2018
11
Recurrent postinfectious glomerulonephritis: an unusual evolution compatible with C3 glomerulopathy. ( 29477994 )
2018
12
C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important Manifestations of Complement System Dysfunction. ( 29594148 )
2018
13
C3 glomerulonephritis secondary to mutations in factors H and I: rapid recurrence in deceased donor kidney transplant effectively treated with eculizumab. ( 29370420 )
2018
14
Statistical Validation of Rare Complement Variants Provides Insights into the Molecular Basis of Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy. ( 29500241 )
2018
15
An Engineered Complement Factor H Construct for Treatment of C3 Glomerulopathy. ( 29588430 )
2018
16
A case of recurrent proliferative glomerulonephritis with monoclonal IgG deposits or de novo C3 glomerulonephritis after kidney transplantation. ( 29968411 )
2018
17
The Complexity of Renal Involvment in IBD-C3 Glomerulopathy in Ulcerative Colitis. ( 29522131 )
2018
18
Prevention of Fatal C3 Glomerulopathy by Recombinant Complement Receptor of the Ig Superfamily. ( 29895552 )
2018
19
Treating C3 glomerulopathy with eculizumab. ( 29329521 )
2018
20
C3 glomerulopathy in NLRP12-related autoinflammatory disorder: case-based review. ( 29951964 )
2018
21
C3 glomerulonephritis and thrombotic microangiopathy of renal allograft after pulmonary infection in a male with concomitant two complement factor I gene variations: a case report. ( 29940891 )
2018
22
Three cases of monoclonal gammopathy of renal significance after kidney transplantation. De novo C3 glomerulopathy. ( 29914760 )
2018
23
Dominant C3 glomerulopathy: new roles for an old actor in renal pathology. ( 29151252 )
2018
24
C3 Glomerulopathy: A New Disease Comes of Age. ( 30077212 )
2018
25
C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic. ( 30077216 )
2018
26
Membranoproliferative glomerulonephritis and C3 glomerulopathy in children: change in treatment modality? A report of a case series. ( 30094012 )
2018
27
Conservative treatment for C3 glomerulopathy and monoclonal Ig. ( 30143070 )
2018
28
Benefit of Eculizumab Compared to Standard of Care Still Unproven in C3 Glomerulopathy. ( 30236630 )
2018
29
Outcome of membranoproliferative glomerulonephritis and C3-glomerulopathy in children and adolescents. ( 30238151 )
2018
30
Both Monoclonal and Polyclonal Immunoglobulin Contingents Mediate Complement Activation in Monoclonal Gammopathy Associated-C3 Glomerulopathy. ( 30333829 )
2018
31
Ravindran A, Fervenza FC, Smith RJH, Sethi S. C3 glomerulopathy associated with monoclonal Ig is a distinct subtype. Kidney Int. 2018;94:178-186. ( 30348291 )
2018
32
Successful Treatment of Posttransplant Recurrent Complement C3 Glomerulopathy with Eculizumab. ( 30367025 )
2018
33
C3 glomerulopathy in children - is there still a place for anti-cellular immunosuppression? ( 30393898 )
2018
34
Kidney Transplantation in C3 Glomerulopathy: A Case Series. ( 30413277 )
2018
35
A Case of Switch from C3 Glomerulonephritis to Proliferative Glomerulonephritis with Monoclonal IgG Deposits. ( 30143498 )
2018
36
Fukushima journal of medical science original articles re-revised-manuscript No-2018-05 predictive factors for poor outcome in pediatric C3 glomerulonephritis. ( 30369521 )
2018
37
Corticosteroid therapy alone for the treatment of C3 glomerulonephritis in association with monoclonal gammopathy‚Ä©. ( 30474593 )
2018
38
Recurrent allograft C3 glomerulonephritis and unsuccessful eculizumab treatment. ( 29097196 )
2018
39
High-dose melphalan and autologous hematopoietic stem cell transplant in patient with C3 glomerulonephritis associated with monoclonal gammopathy‚Ä©. ( 29249234 )
2018
40
C3 glomerulonephritis associated with monoclonal gammopathy of renal significance: case report. ( 29884135 )
2018
41
C3-glomerulopathy and MGUS: the skin beyond the kidney. ( 29272460 )
2017
42
Acquired partial lipodystrophy and C3 glomerulopathy: Dysregulation of the complement system as a common pathogenic mechanism. ( 29279276 )
2017
43
C4 Nephritic Factors in C3 Glomerulopathy: A Case Series. ( 28838767 )
2017
44
C3 glomerulopathy associated to multiple myeloma successfully treated by autologous stem cell transplant. ( 28356669 )
2017
45
Treatment of B-cell disorder improves renal outcome of patients with monoclonal gammopathy-associated C3 glomerulopathy. ( 28069603 )
2017
46
C3 glomerulopathy and eculizumab: a report on four paediatric cases. ( 28236143 )
2017
47
Case report: C3 glomerulopathy advancing atypical hemolytic uremic syndrome. ( 29150227 )
2017
48
Atypical Plasmacytic Proliferation in a Case of C3 Glomerulopathy: Pathophysiology Demystified. ( 28210641 )
2017
49
Crescentic C3 glomerulopathy with acquired partial lipodystrophy: An unusual cause of rapidly progressive renal failure. ( 28631660 )
2017
50
Anti-Factor B and Anti-C3b Autoantibodies in C3 Glomerulopathy and Ig-Associated Membranoproliferative GN. ( 28096309 )
2017

Variations for C3 Glomerulopathy

Expression for C3 Glomerulopathy

Search GEO for disease gene expression data for C3 Glomerulopathy.

Pathways for C3 Glomerulopathy

GO Terms for C3 Glomerulopathy

Cellular components related to C3 Glomerulopathy according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.55 C3 CFB CFHR1 CFHR5 CFP
2 protein-containing complex GO:0032991 9.43 C3 CFHR1 CFHR5
3 extracellular space GO:0005615 9.35 C3 CFB CFHR1 CFHR3 CFP
4 blood microparticle GO:0072562 8.92 C3 CFB CFHR1 CFHR3

Biological processes related to C3 Glomerulopathy according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 proteolysis GO:0006508 9.58 C3 CFB CFP
2 innate immune response GO:0045087 9.54 C3 CFB CFP
3 complement activation GO:0006956 9.46 C3 CFB CFHR1 CFP
4 regulation of complement activation GO:0030449 9.35 C3 CFB CFHR1 CFHR5 CFP
5 negative regulation of protein binding GO:0032091 9.32 CFHR1 CFHR5
6 positive regulation of cytolysis GO:0045919 9.26 CFHR1 CFHR5
7 complement activation, alternative pathway GO:0006957 8.92 C3 CFB CFHR5 CFP

Molecular functions related to C3 Glomerulopathy according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase activity GO:0004252 8.8 C3 CFB CFP

Sources for C3 Glomerulopathy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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