Aliases & Classifications for C3 Glomerulopathy

MalaCards integrated aliases for C3 Glomerulopathy:

Name: C3 Glomerulopathy 25 26
C3 Glomerulonephritis 26 30 74
C3g 25 26
Membranoproliferative Glomerulonephritis, Type Ii 74
Membranoproliferative Glomerulonephritis Type Ii 26
Glomerulonephritis with Dominant C3 25
Dense Deposit Disease 26
Ddd/mpgnii 26
Ddd 26

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Summaries for C3 Glomerulopathy

Genetics Home Reference : 26 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 dense deposit disease and glomerulonephritis. 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 106)
# Related Disease Score Top Affiliating Genes
1 dense deposit disease 30.1 C3 CFB CFHR5
2 glomerulonephritis 30.1 C3 CFHR5 CFP
3 membranoproliferative glomerulonephritis 29.5 C3 CFB CFHR5 CFP
4 hemolytic-uremic syndrome 29.4 C3 CFB CFHR1 CFHR3 CFHR5
5 hemolytic uremic syndrome, atypical 1 28.7 C3 CFB CFHR1 CFHR3 CFHR5 CFP
6 macular degeneration, age-related, 1 28.5 C3 CFB CFHR1 CFHR3 CFHR5
7 dowling-degos disease 1 12.1
8 non-immunoglobulin-mediated membranoproliferative glomerulonephritis 11.8
9 dowling-degos disease 11.4
10 acne inversa, familial, 2, with or without dowling-degos disease 11.4
11 degenerative disc disease 11.4
12 dowling-degos disease 2 11.3
13 adrenal cortical carcinoma 10.5
14 atrioventricular block 10.5
15 glomerulopathy with fibronectin deposits 2 10.4
16 fibrillary glomerulonephritis 10.4
17 immunotactoid glomerulopathy 10.4
18 collagen type iii glomerulopathy 10.4
19 adrenocortical carcinoma, hereditary 10.4
20 adrenal cortical adenocarcinoma 10.4
21 atrial standstill 1 10.3
22 conn's syndrome 10.3
23 hydronephrosis 10.2
24 sick sinus syndrome 10.2
25 spondyloocular syndrome 10.2
26 leukemia 10.2
27 myeloid leukemia 10.2
28 lipodystrophy, partial, acquired 10.1
29 complement factor h deficiency 10.1
30 mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome 10.1
31 kidney disease 10.1
32 atrial fibrillation 10.1
33 adrenal carcinoma 10.1
34 niemann-pick disease, type c1 10.0
35 hepatitis 10.0
36 niemann-pick disease 10.0
37 hepatitis c 10.0
38 cryoglobulinemia 10.0
39 proliferative glomerulonephritis 10.0
40 neurofibromatosis, type ii 10.0
41 malignant atrophic papulosis 10.0
42 third-degree atrioventricular block 10.0
43 hidradenitis suppurativa 10.0
44 hypertrophic cardiomyopathy 10.0
45 crescentic glomerulonephritis 10.0
46 monoclonal gammopathy of uncertain significance 10.0
47 breast cancer 10.0
48 cervical squamous cell carcinoma 10.0
49 lymphoma 10.0
50 generalized epilepsy with febrile seizures plus 10.0

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 Immunoglobulin A Phase 2
5 Antibodies Phase 2,Not Applicable
6 Antigen-Antibody Complex Phase 2,Not Applicable
7 Immunoglobulins Phase 2,Not Applicable
8 Antibodies, Monoclonal Phase 2
9 Immunosuppressive Agents Phase 1
10 Complement C3 Phase 1
11 Complement Inactivating Agents Phase 1

Interventional clinical trials:

(show all 13)
# 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 Study on Efficacy and Safety of LNP023 in C3 Glomerulopathy Patients Transplanted and Not Transplanted Recruiting NCT03832114 Phase 2 LNP023
6 Daratumumab in Treatment of PGNMID and C3 GN Recruiting NCT03095118 Phase 2 Daratumumab
7 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
8 Phase II Study Assessing Safety and Efficacy of APL-2 in Glomerulopathies Recruiting NCT03453619 Phase 2 APL-2
9 OL Extension Study of LNP023 in C3G Not yet recruiting NCT03955445 Phase 2 LNP023
10 First-In-Human Clinical Study of the C3 Complement Inhibitor AMY- 101 in Healthy Male Volunteers Completed NCT03316521 Phase 1 AMY-101
11 Clinical Trial of CDX-1135 in Pediatric and Adult Patients With Dense Deposit Disease Terminated NCT01791686 Phase 1 CDX-1135
12 TP10 Use in Patients With C3 Glomerulopathy (C3G) Withdrawn NCT02302755 Phase 1 TP10
13 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 30

Anatomical Context for C3 Glomerulopathy

MalaCards organs/tissues related to C3 Glomerulopathy:

42
Kidney, Skin, Testes, B Cells, Colon, Breast, Myeloid

Publications for C3 Glomerulopathy

Articles related to C3 Glomerulopathy:

(show top 50) (show all 163)
# Title Authors Year
1
Three cases of monoclonal gammopathy of renal significance after kidney transplantation. De novo C3 glomerulopathy. ( 29914760 )
2019
2
C3 glomerulopathy in children: Is there still a place for anti-cellular immunosuppression? ( 30393898 )
2019
3
Kidney Transplantation in C3 Glomerulopathy: A Case Series. ( 30413277 )
2019
4
C3 glomerulopathy - understanding a rare complement-driven renal disease. ( 30692664 )
2019
5
Diagnosis and treatment of C3 glomerulopathy in a center of expertise. ( 30774098 )
2019
6
C3 glomerulopathy, a new but still evolving entity. ( 30774099 )
2019
7
Factor B and C4b2a Autoantibodies in C3 Glomerulopathy. ( 31024533 )
2019
8
Glomerular C4d Staining Does Not Exclude a C3 Glomerulopathy. ( 31080925 )
2019
9
Corticosteroid therapy alone for the treatment of C3 glomerulonephritis in association with monoclonal gammopathy‚Ä©. ( 30474593 )
2019
10
Widening spectrum of renal involvement in psoriasis: First reported case of C3 glomerulonephritis in a psoriatic patient. ( 30804292 )
2019
11
Clinical course and outcome after kidney transplantation in patients with C3 glomerulonephritis due to CFHR5 nephropathy. ( 30844074 )
2019
12
A novel mutation in complement 2 accompanied by susceptibility variants in C3 glomerulonephritis: A case study. ( 31014550 )
2019
13
Case report: C3 glomerulopathy advancing atypical hemolytic uremic syndrome. ( 29150227 )
2018
14
Dominant C3 glomerulopathy: new roles for an old actor in renal pathology. ( 29151252 )
2018
15
C3-glomerulopathy and MGUS: the skin beyond the kidney. ( 29272460 )
2018
16
Acquired partial lipodystrophy and C3 glomerulopathy: Dysregulation of the complement system as a common pathogenic mechanism. ( 29279276 )
2018
17
C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy. ( 29310824 )
2018
18
Mycophenolate Mofetil in Combination with Steroids for Treatment of C3 Glomerulopathy: A Case Series. ( 29326307 )
2018
19
Treating C3 glomerulopathy with eculizumab. ( 29329521 )
2018
20
Patterns of Clinical Response to Eculizumab in Patients With C3 Glomerulopathy. ( 29429752 )
2018
21
Recurrent postinfectious glomerulonephritis: an unusual evolution compatible with C3 glomerulopathy. ( 29477994 )
2018
22
Statistical Validation of Rare Complement Variants Provides Insights into the Molecular Basis of Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy. ( 29500241 )
2018
23
Evolving complexity of complement-related diseases: C3 glomerulopathy and atypical haemolytic uremic syndrome. ( 29517501 )
2018
24
The Complexity of Renal Involvment in IBD-C3 Glomerulopathy in Ulcerative Colitis. ( 29522131 )
2018
25
Genetic analysis of the complement pathway in C3 glomerulopathy. ( 29566171 )
2018
26
An Engineered Complement Factor H Construct for Treatment of C3 Glomerulopathy. ( 29588430 )
2018
27
C3 glomerulopathy in cystic fibrosis: a case report. ( 29592796 )
2018
28
C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important Manifestations of Complement System Dysfunction. ( 29594148 )
2018
29
Eculizumab as salvage therapy for recurrent monoclonal gammopathy-induced C3 glomerulopathy in a kidney allograft. ( 29724174 )
2018
30
C3 glomerulopathy associated with monoclonal Ig is a distinct subtype. ( 29729982 )
2018
31
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
32
Prevention of Fatal C3 Glomerulopathy by Recombinant Complement Receptor of the Ig Superfamily. ( 29895552 )
2018
33
Autoimmune abnormalities of the alternative complement pathway in membranoproliferative glomerulonephritis and C3 glomerulopathy. ( 29948306 )
2018
34
C3 glomerulopathy in NLRP12-related autoinflammatory disorder: case-based review. ( 29951964 )
2018
35
C3 Glomerulopathy: A New Disease Comes of Age. ( 30077212 )
2018
36
C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic. ( 30077216 )
2018
37
Membranoproliferative glomerulonephritis and C3 glomerulopathy in children: change in treatment modality? A report of a case series. ( 30094012 )
2018
38
Conservative treatment for C3 glomerulopathy and monoclonal Ig. ( 30143070 )
2018
39
Benefit of Eculizumab Compared to Standard of Care Still Unproven in C3 Glomerulopathy. ( 30236630 )
2018
40
Outcome of membranoproliferative glomerulonephritis and C3-glomerulopathy in children and adolescents. ( 30238151 )
2018
41
Both Monoclonal and Polyclonal Immunoglobulin Contingents Mediate Complement Activation in Monoclonal Gammopathy Associated-C3 Glomerulopathy. ( 30333829 )
2018
42
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
43
Successful Treatment of Posttransplant Recurrent Complement C3 Glomerulopathy with Eculizumab. ( 30367025 )
2018
44
Predictive factors for poor outcome in pediatric C3 glomerulonephritis. ( 30369521 )
2018
45
C3 Glomerulonephritis With Multiple Mutations in Complement Factor H. ( 30595568 )
2018
46
C3 glomerulonephritis associated with monoclonal gammopathy of renal significance: case report. ( 29884135 )
2018
47
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
48
A case of recurrent proliferative glomerulonephritis with monoclonal IgG deposits or de novo C3 glomerulonephritis after kidney transplantation. ( 29968411 )
2018
49
A Case of Switch from C3 Glomerulonephritis to Proliferative Glomerulonephritis with Monoclonal IgG Deposits. ( 30143498 )
2018
50
Recurrent allograft C3 glomerulonephritis and unsuccessful eculizumab treatment. ( 29097196 )
2018

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 innate immune response GO:0045087 9.54 C3 CFB CFP
2 complement activation GO:0006956 9.46 C3 CFB CFHR1 CFP
3 regulation of complement activation GO:0030449 9.35 C3 CFB CFHR1 CFHR5 CFP
4 negative regulation of protein binding GO:0032091 9.32 CFHR1 CFHR5
5 positive regulation of cytolysis GO:0045919 9.26 CFHR1 CFHR5
6 proteolysis GO:0006508 9.1 CFB
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 8.32 CFB

Sources for C3 Glomerulopathy

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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