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MCID: CLC001
MIFTS: 49

Calciphylaxis

Categories: Blood diseases, Bone diseases, Cardiovascular diseases, Metabolic diseases, Rare diseases, Skin diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Calciphylaxis

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MalaCards integrated aliases for Calciphylaxis:

Name: Calciphylaxis 12 75 53 59 44 15 72
Idiopathic Calciphylaxis 53

Characteristics:

Orphanet epidemiological data:

59
calciphylaxis
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: Adult; Age of death: adult;

Classifications:

MalaCards categories:
Global: Rare diseases Metabolic diseases
Anatomical: Cardiovascular diseases Bone diseases Skin diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 34
Orphanet: 59  
Rare circulatory system diseases
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:4734
MeSH 44 D002115
NCIt 50 C84607
ICD10 via Orphanet 34 E83.5
UMLS via Orphanet 73 C0006666
Orphanet 59 ORPHA280062
UMLS 72 C0006666
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Summaries for Calciphylaxis

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NIH Rare Diseases : 53 Calciphylaxis is a disease in which blood vessels (veins and arteries) become blocked by a build-up of calcium in the walls of the vessels, preventing blood from flowing to the skin or internal organs. The lack of blood flow (ischemia) damages healthy tissue and causes it to die (necrosis). The most obvious and frequent symptom of calciphylaxis is damage to the skin, as ulcers can develop and become infected easily. Calciphylaxis can also affect fat tissue, internal organs, and skeletal muscle, causing infections, pain, and organ failure. These symptoms are often irreversible, and many individuals with calciphylaxis may not survive more than a few months after they are diagnosed due to infection that spreads throughout the body (sepsis), or organ failure. The exact cause of calciphylaxis is unknown. Treatments may include medications to reduce pain, antibiotics to treat infections, and various approaches to preventing the development or worsening of this condition.

MalaCards based summary : Calciphylaxis, also known as idiopathic calciphylaxis, is related to end stage renal failure and hyperparathyroidism. An important gene associated with Calciphylaxis is ALB (Albumin), and among its related pathways/superpathways are Ca, cAMP and Lipid Signaling and Transcription_Role of VDR in regulation of genes involved in osteoporosis. The drugs Capsaicin and Phylloquinone have been mentioned in the context of this disorder. Affiliated tissues include skin, kidney and bone, and related phenotypes are skin ulcer and gangrene

Wikipedia : 75 Calciphylaxis, also known as calcific uremic arteriolopathy (CUA) or "Grey Scale", is a rare painful... more...

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Related Diseases for Calciphylaxis

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

(show top 50) (show all 249)
# Related Disease Score Top Affiliating Genes
1 end stage renal failure 30.7 PTH FN1 ALB
2 hyperparathyroidism 30.3 VDR PTH FGF23 CASR
3 osteitis fibrosa 30.2 PTH CASR
4 metabolic acidosis 30.0 PTH ALB
5 nephrolithiasis, calcium oxalate 29.9 VDR CASR
6 arterial calcification of infancy 29.9 NT5E FGF23
7 parathyroid adenoma 29.8 VDR PTH CASR
8 nephrocalcinosis 29.7 PTH CASR ALB
9 hypophosphatemia 29.7 PTH FGF23
10 hypoparathyroidism 29.6 PTH FGF23 CASR
11 hyperphosphatemia 29.6 VDR PTH FGF23 CASR
12 kidney disease 29.5 VDR PTH FGF23 CASR ALB
13 bone resorption disease 29.4 VDR PTH
14 rickets 29.3 VDR PTH FGF23
15 uremia 29.3 VDR PTH CASR ALB
16 bone disease 29.1 VDR PTH FGF23 CASR
17 primary hyperparathyroidism 29.0 VDR PTH FGF23 CASR ALB
18 renal osteodystrophy 28.8 VDR PTH FGF23 CASR
19 secondary hyperparathyroidism of renal origin 28.7 VDR PTH FGF23 CASR ALB
20 mineral metabolism disease 28.6 VDR PTH FGF23 CASR
21 osteoporosis 28.5 VDR PTH FGF23 CASR
22 hypocalcemia, autosomal dominant 1 28.1 VDR PTH FGF23 CASR ALB
23 visceral calciphylaxis 12.3
24 calciphylaxis cutis 12.3
25 secondary hyperparathyroidism 10.8
26 arterial calcification, generalized, of infancy, 1 10.7
27 chronic kidney disease 10.7
28 vasculitis 10.5
29 panniculitis 10.4
30 calcinosis 10.4
31 systemic lupus erythematosus 10.4
32 vascular disease 10.3
33 lupus erythematosus 10.3
34 purpura 10.3
35 acute kidney failure 10.3
36 poems syndrome 10.3
37 protein s deficiency 10.3
38 connective tissue disease 10.3
39 liver cirrhosis 10.2
40 hypocalciuric hypercalcemia, familial, type i 10.2 PTH CASR
41 hypocalciuric hypercalcemia, familial, type ii 10.2 PTH CASR
42 hypocalciuric hypercalcemia, familial, type iii 10.2 PTH CASR
43 hypoparathyroidism, familial isolated 10.2 PTH CASR
44 thyroid crisis 10.2 PTH ALB
45 gastrointestinal ulceration, recurrent, with dysfunctional platelets 10.2
46 peripheral vascular disease 10.2
47 polyneuropathy 10.2
48 toxic shock syndrome 10.2
49 familial isolated hyperparathyroidism 10.2 PTH CASR
50 rheumatoid arthritis 10.2

Graphical network of the top 20 diseases related to Calciphylaxis:



Diseases related to Calciphylaxis
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Symptoms & Phenotypes for Calciphylaxis

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Human phenotypes related to Calciphylaxis:

59 32 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 skin ulcer 59 32 hallmark (90%) Very frequent (99-80%) HP:0200042
2 gangrene 59 32 hallmark (90%) Very frequent (99-80%) HP:0100758
3 cellulitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0100658
4 sepsis 59 32 hallmark (90%) Very frequent (99-80%) HP:0100806
5 cutis marmorata 59 32 hallmark (90%) Very frequent (99-80%) HP:0000965
6 hyperphosphatemia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002905
7 stage 5 chronic kidney disease 59 32 hallmark (90%) Very frequent (99-80%) HP:0003774
8 secondary hyperparathyroidism 59 32 hallmark (90%) Very frequent (99-80%) HP:0000867
9 abnormality of skin physiology 59 32 hallmark (90%) Very frequent (99-80%) HP:0011122
10 arterial calcification 59 32 hallmark (90%) Very frequent (99-80%) HP:0003207
11 ectopic ossification 59 32 hallmark (90%) Very frequent (99-80%) HP:0011986
12 abnormality of metabolism/homeostasis 59 Very frequent (99-80%)

GenomeRNAi Phenotypes related to Calciphylaxis according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased melanin production GR00056-A 8.62 FGF23 NT5E

MGI Mouse Phenotypes related to Calciphylaxis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.87 ALB CASR FGF23 FN1 NT5E PTH
2 cardiovascular system MP:0005385 9.85 FGF23 FN1 NT5E PTH VDR
3 hematopoietic system MP:0005397 9.85 CASR FGF23 FN1 NT5E PTH VDR
4 endocrine/exocrine gland MP:0005379 9.8 ALB CASR FGF23 PTH VDR
5 digestive/alimentary MP:0005381 9.73 ALB CASR FGF23 VDR
6 mortality/aging MP:0010768 9.63 ALB CASR FGF23 FN1 PTH VDR
7 limbs/digits/tail MP:0005371 9.56 FGF23 FN1 PTH VDR
8 renal/urinary system MP:0005367 9.35 ALB CASR FGF23 NT5E VDR
9 skeleton MP:0005390 9.02 CASR FGF23 FN1 PTH VDR
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Drugs & Therapeutics for Calciphylaxis

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

(show all 26)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Capsaicin Approved Phase 4 404-86-4 1548943
2
Phylloquinone Approved, Investigational Phase 4 84-80-0
3
Menadione Approved, Nutraceutical Phase 4 58-27-5 4055
4 Menaquinone Investigational Phase 4 1182-68-9
5 Peripheral Nervous System Agents Phase 4
6 Dermatologic Agents Phase 4
7 Antipruritics Phase 4
8 Micronutrients Phase 4
9 Trace Elements Phase 4
10 Vitamins Phase 4
11 Coagulants Phase 4
12 Antifibrinolytic Agents Phase 4
13 naphthoquinone Phase 4
14 Hemostatics Phase 4
15 Nutrients Phase 4
16 Vitamin K Phase 4
17 Vitamin K 2 Phase 4
18 Vitamin MK 7 Phase 4
19 Vitamin K 1 Phase 3
20 Pharmaceutical Solutions Phase 2
21
Warfarin Approved 81-81-2 6691 54678486
22
Cisplatin Approved 15663-27-1 84093 441203 2767
23
carbamide peroxide Approved 124-43-6
24
Calcium Approved, Nutraceutical 7440-70-2 271
25 Calcium, Dietary
26 Anesthetics

Interventional clinical trials:

(show all 21)
# Name Status NCT ID Phase Drugs
1 The Role of Qutenza (Topical Capsaicin 8%) in Treating Neuropathic Pain From Critical Ischaemia in Patients With End-stage Renal Failure Unknown status NCT01704339 Phase 4 QUTENZA
2 Risk Factors for Vascular Calcifications in Hemodialysis Patients: to What Extent is Vitamin K2 Deficiency Involved? Completed NCT02876354 Phase 4 Menaquinone
3 A Phase 3, Intravenous Sodium Thiosulfate for Acute Calciphylaxis Treatment: A Multicenter, Randomized, Double-blind, Placebo-controlled, Clinical Trial Recruiting NCT03150420 Phase 3 Sodium Thiosulfate;Placebo-Normal Saline
4 Vitamin K1 to Slow Progression of Vascular Calcification in Hemodialysis Patients Active, not recruiting NCT01742273 Phase 3 Vitamin K1
5 Randomized Double Blind Placebo Controlled Trial of Sodium Thiosulfate for the Treatment of Pain Associated With Calcific Uremic Arteriolopathy Followed by a Single Armed Extension Phase to Prospectively Observe Lesion Progression in the Presence of Sodium Thiosulfate Treatment Terminated NCT02527213 Phase 3 Sodium thiosulfate;Placebo
6 Phase 2 Open Label Single Arm Repeat Dose Study to Assess the Effect of SNF472 on Wound Healing in Uraemic Calciphylaxis Patients Completed NCT02790073 Phase 2 SNF472
7 Efficacy and Tolerance of Sodium Thiosulfate Injection After Ultrasound-guided Percutaneous Irrigation of Calcific Tendinopathy of the Rotator Cuff Completed NCT02538939 Phase 2 Sodium thiosulfate
8 Novel Drug Delivery of Sodium Thiosulfate for Calcinosis Associated With Adult and Juvenile Dermatomyositis Completed NCT01572844 Phase 2 Sodium thiosulfate
9 A Randomized, Open Label, Four Week, Pharmacodynamic/Pharmacokinetic, Efficacy, and Safety Study of Lunacalcipol (CTA018) Injection in Subjects With Stage 5 Chronic Kidney Disease With Secondary Hyperparathyroidism on Hemodialysis Withdrawn NCT01453634 Phase 2 Lunacalcipol 180;Lunicalcipol 270
10 A Pilot Study to Evaluate Topical Sodium Thiosulfate Therapy for Calcinosis Cutis (STS-CALC) Withdrawn NCT01918904 Phase 2 Sodium thiosulfate;Placebo
11 Evaluation of Efficacy of Lanthanum Carbonate (Fosrenol) in Patients With Calciphylaxis Completed NCT01289626 Phase 1 lanthanum carbonate;Lanthanum for calciphylaxis
12 Calciphylaxis : Population, Risk Factors, Diagnostic Practice, Therapeutic and Outcome : a Multicentric, Retrospective Cohort Unknown status NCT02854046
13 National Follow-up of Dialysis Patients With Sodium Thiosufate-treated Calciphylaxis Under Temporary Use Authorization Unknown status NCT03146793 Sodium Thiosulfate
14 Pathophysiology of Martorell Hypertensive Ischemic Leg Ulcer (HYTILU): A Comparison of Martorell HYTILU, Calciphylaxis and Venous Ulcer (Control) Unknown status NCT01578382
15 Partners Calciphylaxis Biobank and Patient Registry Recruiting NCT03032835
16 European Calciphylaxis Registry Network Recruiting NCT02635373
17 Evaluation of Vitamin K Supplementation for Calcific Uremic Arteriolopathy Recruiting NCT02278692
18 Impact of Parathyroidectomy on Vascular Calcification and Clinical Outcome in Hemodialysis Patients Recruiting NCT03724188
19 Observational Follow-up to ST-001 Calciphylaxis Pain Treatment With Intravenous Sodium Thiosulfate Terminated NCT03319914
20 A Pilot, Randomized, Self-controlled Study of the Effects of Intratympanic Sodium Thiosulfate on the Degree of Hearing Loss in Patients Receiving Cisplatin Therapy Terminated NCT01369641 Sodium Thiosulfate (STS);Cisplatin
21 Modulation of Gut Microbiota in End-stage Renal Disease: A Pilot Study Withdrawn NCT02568891

Search NIH Clinical Center for Calciphylaxis

Cochrane evidence based reviews: calciphylaxis

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Genetic Tests for Calciphylaxis

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Anatomical Context for Calciphylaxis

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MalaCards organs/tissues related to Calciphylaxis:

41
Skin, Kidney, Bone, Skeletal Muscle, Breast, Liver, Heart
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Publications for Calciphylaxis

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Articles related to Calciphylaxis:

(show top 50) (show all 1191)
# Title Authors PMID Year
1
Refractory secondary hyperparathyroidism in waiting list for parathyroidectomy: who we should operate first in a quaternary hospital in Brazil regarding survival. 38
30942649 2019
2
Clinicopathologic Features and Calcium Deposition Patterns in Calciphylaxis: Comparison With Gangrene, Peripheral Artery Disease, Chronic Stasis, and Thrombotic Vasculopathy. 38
31192861 2019
3
Revisiting therapeutic options for calciphylaxis. 38
31169527 2019
4
Penile calciphyilaxis treated with daily Tadalafil: Case report. 38
31372344 2019
5
Resolution of acenocoumarol-associated calciphylaxis with drug withdrawal. 38
30790279 2019
6
Parathyroidectomy as a Cure for Calciphylaxis in a Non-Dialysis Chronic Kidney Disease Patient? 38
31395848 2019
7
Improvement in wound healing, pain, and quality of life after 12 weeks of SNF472 treatment: a phase 2 open-label study of patients with calciphylaxis. 38
31401795 2019
8
Dermatologic Urgencies and Emergencies: What Every Pathologist Should Know. 38
30785787 2019
9
Calciphylaxis in a dialysis patient treated by intralesional and systemic sodium thiosulphate on top of multifactorial intervention. 38
31384447 2019
10
Oral Anticoagulation for Atrial Fibrillation Thromboembolism Prophylaxis in the Chronic Kidney Disease Population: the State of the Art in 2019. 38
31165356 2019
11
The use of cinacalcet for the treatment of calciphylaxis in patients with chronic kidney disease: A comprehensive review. 38
30666627 2019
12
Calciphylaxis and nephrogenic fibrosing dermopathy with pseudoxanthoma elasticum-like changes: Successful treatment with sodium thiosulfate. 38
30663093 2019
13
Hemodialysis coupled with rheopheresis in calciphylaxis: A winning combination. 38
31322751 2019
14
Do I Have Calciphylaxis? 38
31116385 2019
15
[The nightmare of calciphylaxis]. 38
31344746 2019
16
Localization, Morphologic Features, and Chemical Composition of Calciphylaxis-Related Skin Deposits in Patients With Calcific Uremic Arteriolopathy. 38
31116362 2019
17
New Debates Surrounding the Histopathological Findings of Calciphylaxis-Do These Give Us New Insights Into Pathogenesis of the Disease? 38
31116348 2019
18
Image-Guided Core-Needle Biopsy for the Diagnosis of Cutaneous Calciphylaxis. 38
31116352 2019
19
Comparison of Safety Profiles of New Oral Anticoagulants with Warfarin Using the Japanese Spontaneous Reporting Database. 38
31054085 2019
20
[Efficacy of sodium thiosulphate in a case of calciphylaxis in a chronic nephropathic ninety-year-old patient on conservative therapy]. 38
31251002 2019
21
The first case of coexistence of calciphylaxis and nephrogenic systemic fibrosis with different localizations in a hemodialysis patient. 38
31182286 2019
22
Warfarin in nonvalvular atrial fibrillation-Time for a change? 38
31209952 2019
23
A Catastrophic Case of Idiopathic Cholesterol Crystal Embolism with Multiple Lethal Complications: A Labyrinth Underneath the Diagnosis of Skin Ulcers in Chronic Kidney Disease Patients. 38
30713332 2019
24
The association of iron overload and development of calciphylaxis. 38
31255363 2019
25
Calciphylaxis: Diagnosis, Pathogenesis, and Treatment. 38
31008757 2019
26
Calcific uremic arteriolopathy (calciphylaxis) calls into question the validity of guidelines of diagnosis and treatment. 38
31075991 2019
27
A persistent penile ulcer in end stage renal disease: It is not always calciphylaxis. 38
31050098 2019
28
Calciphylaxis After Kidney Transplant. 38
31338270 2019
29
Calciphylaxis: Diagnosis, Pathogenesis, and Treatment. 38
31008761 2019
30
Hypercalcemia associated with sodium thiosulfate treatment in a calciphylaxis patient. 38
31111826 2019
31
Breakthrough invasive Trichosporon asahii infection in an uremic patient with systemic calciphylaxis complicating necrotizing fasciitis during echinocandin therapy for C. tropicalis. 38
31023578 2019
32
Phenprocoumon based anticoagulation is an underestimated factor in the pathogenesis of calciphylaxis. 38
30940121 2019
33
A rare clinical case of penile gangrene in a patient with chronic renal failure and diabetes mellitus. 38
30805296 2019
34
Calciphylaxis as A Rare Cause of A Chronic Wound in An 83-Year-Old Woman. 38
30889873 2019
35
Non-uremic calciphylaxis: a rare and late adverse reaction of warfarin. 38
30834840 2019
36
A unique clinical and histologic presentation of catastrophic systemic calciphylaxis in a nonuremic patient with systemic lupus erythematosus. 38
30854415 2019
37
Pulmonary Calcifications: Is it an Entity in the New World Dialysis Patient? 38
30983755 2019
38
Digital Gangrene in a Dialysis Patient Due to Calciphylaxis and Systemic Polyarteritis Nodosa: A Diagnostic Dilemma. 38
30983759 2019
39
Successful treatment of extensive uremic calciphylaxis with intravenous sodium thiosulfate and its potential in treating various diseases of pathologic calcification. 38
30901068 2019
40
Non-uraemic calciphylaxis successfully treated with pamidronate infusion. 38
30393969 2019
41
Demographics, Comorbid Conditions, and Outcomes of Patients With Nonuremic Calciphylaxis. 38
30601879 2019
42
Bilateral Mastodynia: An Unusual Presentation of Calciphylaxis. 38
30753809 2019
43
Palliative Care Use and Patterns of End-of-Life Care in Hospitalized Patients With Calciphylaxis. 38
30399415 2019
44
Treatment of Calciphylaxis in CKD: A Systematic Review and Meta-analysis. 38
30775620 2019
45
Management of calciphylaxis in a burn center: A case series and review of the literature. 38
30322738 2019
46
Hans Selye and his studies on the role of mast cells in calciphylaxis and calcergy. 38
30456595 2019
47
Glans penis necrosis caused by calcific uremic arteriolopathy. 38
30865408 2019
48
Non-uremic calciphylaxis in a patient with multiple rheumatologic diseases. 38
30865406 2019
49
Calciphylaxis-as a drug induced adverse event. 38
30574812 2019
50
The Effectiveness of Topical Cerium Nitrate-Silver Sulfadiazine Application on Overall Outcome in Patients with Calciphylaxis. 38
30605905 2019
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Variations for Calciphylaxis

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Expression for Calciphylaxis

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Search GEO for disease gene expression data for Calciphylaxis.
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Pathways for Calciphylaxis

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Pathways related to Calciphylaxis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Ca, cAMP and Lipid Signaling 4
11.44 VDR CASR ALB
2
Transcription_Role of VDR in regulation of genes involved in osteoporosis 22
11.02 VDR PTH
3
Parathyroid hormone synthesis, secretion and action 37
10.95 VDR PTH FGF23 CASR
4
Development_Hedgehog and PTH signaling pathways in bone and cartilage development 22
10.91 VDR PTH
5
Osteoblast Signaling 1
10.4 PTH FGF23
6
Vitamin D Metabolism 1
9.96 VDR PTH
Sources

GO Terms for Calciphylaxis

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Cellular components related to Calciphylaxis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 platelet alpha granule lumen GO:0031093 8.96 FN1 ALB
2 endoplasmic reticulum lumen GO:0005788 8.8 FN1 FGF23 ALB

Biological processes related to Calciphylaxis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.63 VDR FN1 CASR
2 post-translational protein modification GO:0043687 9.61 FN1 FGF23 ALB
3 cellular protein metabolic process GO:0044267 9.58 FN1 FGF23 ALB
4 cellular calcium ion homeostasis GO:0006874 9.43 VDR PTH CASR
5 vitamin D metabolic process GO:0042359 9.37 VDR FGF23
6 cellular response to vitamin D GO:0071305 9.32 FGF23 CASR
7 response to fibroblast growth factor GO:0071774 9.16 PTH CASR
8 phosphate ion homeostasis GO:0055062 8.96 PTH FGF23
9 positive regulation of vitamin D 24-hydroxylase activity GO:0010980 8.62 VDR FGF23

Molecular functions related to Calciphylaxis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 chaperone binding GO:0051087 8.96 FN1 ALB
2 drug binding GO:0008144 8.32 ALB
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Sources for Calciphylaxis

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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 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 TGDB
71 Tocris
72 UMLS
73 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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