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LCDD
MCID: LGH004
MIFTS: 40

Light Chain Deposition Disease (LCDD)

Categories: Blood diseases, Immune diseases, Nephrological diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Light Chain Deposition Disease

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MalaCards integrated aliases for Light Chain Deposition Disease:

Name: Light Chain Deposition Disease 52 58
Light-Chain Deposition Disease 52
Light Chain Disease 71
Lcdd 58

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Nephrological diseases Blood diseases Immune diseases
See all MalaCards categories (disease lists)
ICD10: 33
Orphanet: 58  
Rare renal diseases
Rare haematological diseases


External Ids:

ICD10 via Orphanet 33 D89.8
UMLS via Orphanet 72 C0238239
Orphanet 58 ORPHA93558
UMLS 71 C0238239
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Summaries for Light Chain Deposition Disease

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NIH Rare Diseases : 52 Light chain deposition disease (LCDD) is a rare condition characterized by the deposition of specific proteins (monoclonal light chains) in the kidneys and other organs . Light chains are used to make antibodies that the body needs to fight infection. People with LCDD make too many light chains, which get deposited in many body tissues . While LCDD can occur in any organ, the kidneys are always involved. Signs and symptoms of LCDD may include protein in the urine ; decreased kidney function; and/or nephrotic syndrome . Rarely, a person with LCDD may have symptoms from cardiac (heart) or liver involvement. The underlying cause of LCDD is unknown. It is often associated with multiple myeloma . LCDD may progress to multiple myeloma, or it may be present with multiple myeloma when it is first diagnosed. The goal of treating LCDD is to slow the production of light chains and their damage to organs. Treatment may include chemotherapy with a drug called Bortezomib ; autologous stem cell transplantation ; immunomodulatory drugs; and/or kidney transplant. If untreated, end-stage renal disease occurs in 70% of cases.

MalaCards based summary : Light Chain Deposition Disease, also known as light-chain deposition disease, is related to diastolic heart failure and cutis laxa. An important gene associated with Light Chain Deposition Disease is ACTC1 (Actin Alpha Cardiac Muscle 1), and among its related pathways/superpathways are Integrin Pathway and Degradation of the extracellular matrix. The drugs Dexamethasone and Dexamethasone acetate have been mentioned in the context of this disorder. Affiliated tissues include kidney, heart and liver.

Wikipedia : 74 Light chain deposition disease (LCDD) is a rare blood cell disease which is characterized by deposition... more...

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Related Diseases for Light Chain Deposition Disease

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Diseases related to Light Chain Deposition Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 201)
# Related Disease Score Top Affiliating Genes
1 diastolic heart failure 29.1 MMP9 MMP2 MMP1
2 cutis laxa 29.1 MMP9 MMP2 MMP1
3 lymphangioleiomyomatosis 28.6 MMP9 MMP2 MMP14 MMP1
4 lacrimal duct defect 12.0
5 amyloidosis 10.7
6 myeloma, multiple 10.7
7 nephrotic syndrome 10.6
8 plasma cell neoplasm 10.5
9 kidney disease 10.4
10 end stage renal failure 10.4
11 al amyloidosis 10.4
12 malignant giant cell tumor 10.4 CD68 ACTC1
13 non-langerhans-cell histiocytosis 10.4 CD68 ACTC1
14 malignant giant cell tumor of soft parts 10.3 CD68 ACTC1
15 dendritic cell tumor 10.3 CD68 ACTC1
16 ischemic fasciitis 10.3 CD68 ACTC1
17 diabetes and deafness, maternally inherited 10.3
18 cholestasis 10.3
19 pseudosarcomatous fibromatosis 10.3 CD68 ACTC1
20 glomerulonephritis 10.3
21 cutaneous fibrous histiocytoma 10.2 CD68 ACTC1
22 acute kidney failure 10.2
23 monoclonal gammopathy of uncertain significance 10.2
24 neuropathy 10.2
25 rare tumor 10.2 MMP2 ACTC1
26 lymphoproliferative syndrome 10.2
27 portal hypertension 10.2
28 plasmacytoma 10.2
29 restrictive cardiomyopathy 10.2
30 congestive heart failure 10.2
31 light and heavy chain deposition disease 10.2
32 heavy chain deposition disease 10.2
33 immunotactoid glomerulopathy 10.2
34 fibromatosis, gingival, 1 10.1 MMP2 MMP1
35 atrial standstill 1 10.1
36 polyneuropathy 10.1
37 peripheral nervous system disease 10.1
38 multicentric osteolysis-nodulosis-arthropathy spectrum 10.1 MMP2 MMP14
39 winchester syndrome 10.1 MMP2 MMP14
40 mid-dermal elastolysis 10.0 MMP7 MMP12
41 lymphoma, mucosa-associated lymphoid type 10.0
42 retinal detachment 10.0
43 pre-eclampsia 10.0
44 fanconi syndrome 10.0
45 membranoproliferative glomerulonephritis 10.0
46 histiocytosis 10.0
47 myopathy 10.0
48 proliferative glomerulonephritis 10.0
49 b-cell lymphoma 10.0
50 chronic kidney disease 10.0

Graphical network of the top 20 diseases related to Light Chain Deposition Disease:



Diseases related to Light Chain Deposition Disease
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Symptoms & Phenotypes for Light Chain Deposition Disease

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Drugs & Therapeutics for Light Chain Deposition Disease

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Drugs for Light Chain Deposition Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 38)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
2
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
3
Bortezomib Approved, Investigational Phase 3 179324-69-7 387447 93860
4
Melphalan Approved Phase 3 148-82-3 4053 460612
5
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
6
Lenalidomide Approved Phase 3 191732-72-6 216326
7
Phenylalanine Approved, Investigational, Nutraceutical Phase 3 63-91-2 6140
8 Antiemetics Phase 3
9 HIV Protease Inhibitors Phase 3
10
protease inhibitors Phase 3
11 Gastrointestinal Agents Phase 3
12 Anti-Inflammatory Agents Phase 3
13 Hormone Antagonists Phase 3
14 Autonomic Agents Phase 3
15 BB 1101 Phase 3
16 Antineoplastic Agents, Hormonal Phase 3
17 Immunosuppressive Agents Phase 3
18 glucocorticoids Phase 3
19 Hormones Phase 3
20 Alkylating Agents Phase 3
21 Immunologic Factors Phase 3
22 Angiogenesis Inhibitors Phase 3
23
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
24
Mesna Approved, Investigational Phase 2 3375-50-6 598
25
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
26
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
27
Lenograstim Approved, Investigational Phase 2 135968-09-1
28 Anti-Bacterial Agents Phase 2
29 Anti-Infective Agents Phase 2
30 Antirheumatic Agents Phase 2
31
Liposomal doxorubicin Phase 2 31703
32 Dexamethasone 21-phosphate Phase 2
33 Antibodies Phase 2
34 Immunoglobulins Phase 2
35 Adjuvants, Immunologic Phase 2
36 Paraproteins Phase 2
37 Myeloma Proteins Phase 2
38
Pomalidomide Approved Phase 1 19171-19-8

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Randomized Phase III Trial of Melphalan and Dexamethasone (MDex) Versus Bortezomib, Melphalan and Dexamethasone (BMDex) for Untreated Patients With Systemic Light-Chain (AL) Amyloidosis Ineligible for Autologous Stem-Cell Transplantation Completed NCT01078454 Phase 3 melphalan;dexamethasone;bortezomib
2 Randomized Phase III Trial of Lenalidomide Versus Observation Alone in Patients With Asymptomatic High-Risk Smoldering Multiple Myeloma Active, not recruiting NCT01169337 Phase 3 Lenalidomide
3 S0115, A Phase II Trial Evaluating Modified High Dose Melphalan (100 mg/m) And Autologous Peripheral Blood Stem Cell Supported Transplant (SCT) For High Risk Patients With Multiple Myeloma And/Or Light Chain Amyloidosis (AL Amyloidosis) (A BMT Study) Completed NCT00064337 Phase 2 cyclophosphamide;dexamethasone;melphalan;thalidomide
4 An Open-label Phase II Study of the Efficacy of Combination Bortezomib-containing Regimens in the Treatment of Newly Diagnosed Patients With t (4; 14) Positive Multiple Myeloma Completed NCT00570180 Phase 2 Bortezomib
5 A Multicenter Phase II Trial of Bortezomib (Velcade), Melphalan, and Dexamethasone (V-MD) in Patients With Symptomatic AL-Amyloidosis or Light Chain Deposition Disease Active, not recruiting NCT00520767 Phase 2 bortezomib;dexamethasone;melphalan
6 High-Dose Melphalan and Autologous Stem Cell Transplantation (HDM/SCT) in Light-Chain Deposition Disease (LCDD) and Immunoglobulin Deposition Disease (IGDD) Terminated NCT00681044 Phase 2 melphalan
7 A Phase 1 Study of Reolysin Alone in Patients With Relapsed or Refractory Multiple Myeloma Completed NCT01533194 Phase 1
8 Phase I Study of Pomalidomide, Bortezomib, and Dexamethasone (PVD) as First-Line Treatment of AL Amyloidosis or Light Chain Deposition Disease Active, not recruiting NCT01728259 Phase 1 pomalidomide;bortezomib;dexamethasone
9 Pilot Study of Bortezomib/Dexamethasone (BD), Followed By Autologous Stem Cell Transplantation and Maintenance Bortezomib/Dexamethasone For the Initial Treatment of Monoclonal Immunoglobulin Deposition Disease (MIDD) Associated With Multiple Myeloma and AL Amyloidosis Completed NCT01383759 Bortezomib/Dexamethasone (BD), Followed By Autologous STC & Maintenance Bortezomib/Dexamethasone
10 Role of Cardiac MR (CMR) In Detecting Features of Early Myocardial Infiltration With Amyloid and Light Chain Deposition Disease in Subjects With Multiple Myeloma Terminated NCT01018927

Search NIH Clinical Center for Light Chain Deposition Disease

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Genetic Tests for Light Chain Deposition Disease

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Anatomical Context for Light Chain Deposition Disease

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MalaCards organs/tissues related to Light Chain Deposition Disease:

40
Kidney, Heart, Liver, Lung, B Cells, Bone, Tongue
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Publications for Light Chain Deposition Disease

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Articles related to Light Chain Deposition Disease:

(show top 50) (show all 372)
# Title Authors PMID Year
1
Isolated light chain deposition disease neuropathy in a patient with multiple myeloma. 61
31566029 2020
2
[Effect of surgery of pulmonary cysts related to immunoglobulin light chain deposits]. 61
32014310 2020
3
Epidemiological and clinical characteristics and outcome of monoclonal gammopathy of renal significance-related lesions in Latin America. 61
31825549 2019
4
Outcomes of autologous stem cell transplant for cardiac AL-amyloidosis and cardiac light chain deposition disease. 61
31795821 2019
5
The pathogenesis of renal injury and treatment in light chain deposition disease. 61
31767034 2019
6
Clinicopathologic Assessment of Monoclonal Immunoglobulin-Associated Renal Disease in the Kidney Allograft. A Retrospective Study and Review of the Literature. 61
31634325 2019
7
The Evolution of Monoclonal Gammopathy of Undetermined Significance in Kidney Transplant Recipients. 61
31723584 2019
8
Monoclonal gammopathy of renal significance with light-chain deposition disease in kidney transplantation. 61
31696857 2019
9
Proteomic evidence of specific IGKV1-8 association with cystic lung light chain deposition disease. 61
30967366 2019
10
Coexistent Amyloid Fibrils in a Patient with Combined Light Chain Deposition Disease and Light Chain Cast Nephropathy. 61
31142970 2019
11
Comparison of two serum free light chain assays for the diagnosis of primary plasma cell malignant proliferative disease. 61
31049418 2019
12
[A case of bronchiectasis due to light chain deposition disease]. 61
30686554 2019
13
Primary idiopathic CNS non-amyloidogenic light chain deposition disease complicated by treatment-resistant focal seizure disorder. 61
30424969 2019
14
Renal amyloidosis complicated by light chain deposition nephropathy: a case report. 61
31934052 2019
15
Structural analysis of urinary light chains and proteomic analysis of hyaline tubular casts in light chain associated kidney disorders. 61
31592189 2019
16
Acute kidney injury on chronic kidney disease: from congestive heart failure to light chain deposition disease and cast nephropathy in multiple myeloma. 61
31531214 2019
17
Bortezomib-Based Chemotherapy with Autologous Stem Cell Transplantation for Monoclonal Gammopathy of Renal Significance: A Case Report and Literature Review. 61
31352451 2019
18
Histopathologic findings in lung biopsies from patients with primary biliary cholangitis. 61
30067952 2018
19
Treatment of multiple myeloma with renal involvement: the nephrologist's view. 61
30524711 2018
20
Lenalidomide as a Beneficial Treatment Option for Renal Impairment Caused by Light Chain Deposition Disease. 61
30101913 2018
21
Unmasking and successful management of light chain deposition disease of kidney in pregnancy: a complex case, mirroring the complex needs of pregnancy with kidney disease in India. 61
30187379 2018
22
Acquired Fanconi syndrome secondary to light chain deposition disease associated with monoclonal gammopathy of renal significance: A case report. 61
30200082 2018
23
Successful treatment of nephrotic syndrome induced by lambda light chain deposition disease using lenalidomide: A case report and review of the literature
. 61
29393843 2018
24
Primary Central Nervous System Lymphoma With Light Chain Deposition Disease (Aggregoma). 61
29280764 2018
25
Phenotypic plasticity of mesenchymal stem cells is crucial for mesangial repair in a model of immunoglobulin light chain-associated mesangial damage. 61
29668344 2018
26
Cutaneous Light Chain Deposition Disease: A Report of 2 Cases and Review of the Literature. 61
28953013 2018
27
Autologous Stem Cell Transplantation for Multiple Myeloma: Single Centre Experience from North India. 61
29622867 2018
28
Light Chain Deposition Disease Associated With Multiple Myeloma Developing in Late Pregnancy. 61
29507277 2018
29
Light Chain Deposition Disease with Bullous Skin Lesions Mimicking Atypical Bullous Pemphigoid. 61
29135016 2018
30
Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture. 61
30756004 2018
31
Primary CNS Nonamyloidogenic Light Chain Deposition Disease: Case Report and Brief Review. 61
28669250 2017
32
A Case Report of Acute Renal Failure as a Result of Light-Chain-Mediated Acute Tubular Interstitial Nephritis in a 30-Year-Old Combat Veteran. 61
29087890 2017
33
Detection and Treatment of Monoclonal Gammopathy of Renal Significance With Progression to Light Chain Deposition Disease. 61
28809601 2017
34
OCULAR MANIFESTATIONS OF MONOCLONAL IMMUNOGLOBULIN LIGHT CHAIN DEPOSITION DISEASE. 61
27315323 2017
35
Clinical characteristics and prognostic factors in multiple myeloma patients with light chain deposition disease. 61
28383130 2017
36
[The value of serum free light chain in differential diagnosis of monoclonal gammopathy of renal significance]. 61
28822451 2017
37
Cardiac light-chain deposition disease relapsing in the transplanted heart. 61
28562083 2017
38
Intracystic hemorrhage in a patient with pulmonary cystic disorder related to light-chain deposition disease. 61
27756655 2017
39
[Clinical features and prognosis of patients with primary light chain deposition disease]. 61
28395454 2017
40
[Light-chain deposition disease is a hematologic problem]. 61
28252625 2017
41
Plasma cell neoplasia after kidney transplantation: French cohort series and review of the literature. 61
28636627 2017
42
The Lung in Dysregulated States of Humoral Immunity. 61
28910817 2017
43
Light-Chain Deposition Disease with Prominent Hepatic Involvement. 61
30755931 2017
44
ATYPICAL MACULOPATHY IN A PATIENT WITH LIGHT CHAIN DEPOSITION DISEASE MIMICKING ADVANCED GEOGRAPHIC ATROPHY. 61
26934302 2017
45
Light Chain Deposition Disease Diagnosed with Laser Micro-dissection, Liquid Chromatography, and Tandem Mass Spectrometry of Nodular Glomerular Lesions. 61
28050001 2017
46
Treatment of idiopathic light chain deposition disease: complete remission with bortezomib and dexamethasone. 61
28001187 2016
47
Renal disease related to Waldenström macroglobulinaemia: incidence, pathology and clinical outcomes. 61
27468978 2016
48
Cystic Lung Diseases: Algorithmic Approach. 61
27180915 2016
49
Bortezomib Based Chemotherapy for Light Chain Deposition Disease. 61
27762125 2016
50
Nodular pulmonary light chain deposition disease. 61
27306574 2016
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Variations for Light Chain Deposition Disease

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Expression for Light Chain Deposition Disease

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Search GEO for disease gene expression data for Light Chain Deposition Disease.
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Pathways for Light Chain Deposition Disease

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Pathways related to Light Chain Deposition Disease according to GeneCards Suite gene sharing:

(show all 21)
# Super pathways Score Top Affiliating Genes
1
Integrin Pathway 63
Show member pathways
 13.14 TNC MMP9 MMP7 MMP2 MMP12 MMP1
2
Degradation of the extracellular matrix 65
Show member pathways
 12.45 TNC MMP9 MMP7 MMP2 MMP14 MMP12
3
TGF-beta Signaling Pathway (WikiPathways) 1
11.86 TNC MMP12 MMP1
4
Colorectal Cancer Metastasis 63
11.85 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
5
Microglia Activation During Neuroinflammation: Overview 64
Show member pathways
 11.83 TNC MMP9 MMP7 MMP2
6
Adhesion 4
11.81 MMP9 MMP2 MMP14
7
Interleukin-4 and 13 signaling 65
11.77 MMP9 MMP2 MMP1
8
Angiogenesis (CST) 4
11.62 TNC MMP9 MMP7 MMP2
9
Bladder cancer 36 1
11.55 MMP9 MMP2 MMP1
10
Photodynamic therapy-induced NF-kB survival signaling 1
Show member pathways
 11.47 MMP9 MMP2 MMP1
11
AGE/RAGE pathway 1
11.44 MMP9 MMP7 MMP2 MMP14
12
Matrix Metalloproteinases 1
Show member pathways
 11.43 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
13
Cell adhesion_Cell-matrix glycoconjugates 22
11.24 TNC MMP9 MMP1
14
Validated transcriptional targets of AP1 family members Fra1 and Fra2 1
11.16 MMP9 MMP2 MMP1
15
A-beta Pathways: Uptake and Degradation 64
11.1 MMP9 MMP2
16
Syndecan-4-mediated signaling events 1
11.08 TNC MMP9
17
Plasma membrane estrogen receptor signaling 1
11 MMP9 MMP2
18
A-beta Pathways: Plaque Formation and APP Metabolism 64
10.94 MMP9 MMP2
19
Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs) 65
10.89 MMP2 MMP1
20
Cell adhesion_ECM remodeling 22
10.89 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
21
Syndecan-1-mediated signaling events 1
10.81 MMP9 MMP7 MMP1
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GO Terms for Light Chain Deposition Disease

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Cellular components related to Light Chain Deposition Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.76 TNC MMP9 MMP7 MMP2 MMP12 MMP1
2 extracellular space GO:0005615 9.61 TNC MMP9 MMP7 MMP2 MMP14 MMP12
3 blood microparticle GO:0072562 9.43 IGKV1-5 IGKC ACTC1
4 extracellular matrix GO:0031012 9.17 TNC MMP9 MMP7 MMP2 MMP14 MMP12

Biological processes related to Light Chain Deposition Disease according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 proteolysis GO:0006508 9.8 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
2 extracellular matrix organization GO:0030198 9.7 TNC MMP9 MMP7 MMP2 MMP14 MMP12
3 cellular protein metabolic process GO:0044267 9.63 TNC MMP2 MMP1
4 leukocyte migration GO:0050900 9.61 MMP1 IGKV1-5 IGKC
5 endodermal cell differentiation GO:0035987 9.54 MMP9 MMP2 MMP14
6 embryo implantation GO:0007566 9.49 MMP9 MMP2
7 cellular response to reactive oxygen species GO:0034614 9.48 MMP9 MMP2
8 response to amyloid-beta GO:1904645 9.43 MMP9 MMP2 MMP12
9 extracellular matrix disassembly GO:0022617 9.43 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
10 tissue remodeling GO:0048771 9.4 MMP2 MMP14
11 collagen catabolic process GO:0030574 9.1 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1

Molecular functions related to Light Chain Deposition Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hydrolase activity GO:0016787 9.88 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
2 zinc ion binding GO:0008270 9.8 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
3 serine-type endopeptidase activity GO:0004252 9.67 MMP9 MMP7 MMP2 MMP1
4 peptidase activity GO:0008233 9.63 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
5 endopeptidase activity GO:0004175 9.56 MMP9 MMP14 MMP12 MMP1
6 metallopeptidase activity GO:0008237 9.43 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
7 metalloendopeptidase activity GO:0004222 9.1 MMP9 MMP7 MMP2 MMP14 MMP12 MMP1
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Sources for Light Chain Deposition Disease

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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