MYD88 Deficiency

A hereditary autosomal recessive disorder caused by mutations in the MYD88 gene that result in susceptibility to severe, life-threatening, recurrent pyogenic BACTERIAL INFECTIONS in affected children, including invasive pneumococcal disease. OMIM: 612260

Also Known As:

MYD88D; Pyogenic Bacterial Infections, Recurrent, Due To MYD88 Deficiency; Recurrent Pyogenic Bacterial Infections Due To MYD88 Deficiency

Networked: 51 relevant articles (1 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Inflammation (Inflammations)
2.	Infections
3.	Reproductive Tract Infections
4.	Ileus
5.	Abdominal Aortic Aneurysm

Experts

1.	Pasparakis, Manolis: 4 articles (10/2021 - 07/2011)
2.	Akira, Shizuo: 3 articles (12/2018 - 05/2004)
3.	Lacey, Carolyn A: 2 articles (01/2021 - 12/2017)
4.	Skyberg, Jerod A: 2 articles (01/2021 - 12/2017)
5.	Polykratis, Apostolos: 2 articles (01/2019 - 01/2018)
6.	Uematsu, Satoshi: 2 articles (12/2018 - 03/2006)
7.	Arditi, Moshe: 2 articles (10/2018 - 09/2008)
8.	Chen, Shuang: 2 articles (10/2018 - 09/2008)
9.	Crother, Timothy R: 2 articles (10/2018 - 09/2008)
10.	van Loo, Geert: 2 articles (09/2014 - 07/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to MYD88 Deficiency:

1.	LigandsIBA 10/01/2013 - "The effects of MyD88 deficiency on disease phenotype in dysferlin-deficient A/J mice: role of endogenous TLR ligands." 09/01/2011 - "These results suggest that MyD88 deficiency may reduce Aβ load by enhancing the phagocytic capability of microglia through fractalkine (the ligand of CX3CR1) signaling and by promoting apoE-mediated clearance of Aβ from the brain. "
2.	Chemokine CX3CL1IBA 09/01/2011 - "These results suggest that MyD88 deficiency may reduce Aβ load by enhancing the phagocytic capability of microglia through fractalkine (the ligand of CX3CR1) signaling and by promoting apoE-mediated clearance of Aβ from the brain. "
3.	Apolipoproteins E (ApoE)IBA 09/01/2011 - "These results suggest that MyD88 deficiency may reduce Aβ load by enhancing the phagocytic capability of microglia through fractalkine (the ligand of CX3CR1) signaling and by promoting apoE-mediated clearance of Aβ from the brain. "
4.	Proteins (Proteins, Gene)FDA Link 03/01/2014 - "Our studies suggest that not only the loss of protein expression but also the defect of Myddosome formation could cause IRAK4 and MyD88 deficiency syndromes. " 01/01/2021 - "Because the adaptor protein myeloid differentiation primary response protein 88 (MyD88) is essential for downstream signaling of most TLRs and IL-1R, we systematically investigated the effect of MyD88 deficiency on murine brain structure during development and on behavior. " 04/01/2014 - "Using mice deficient in either TLR adapter proteins MyD88 or TRIF we discovered that MyD88 deficiency drastically reduced neutrophil and macrophage infiltration in the kidney, whereas TRIF deficiency decreased T cell infiltrates. " 09/01/2011 - "Because the adapter protein MyD88 is essential for the downstream signaling of all TLRs, except TLR3, we investigated the effects of MyD88 deficiency (MyD88(-/-)) on Aβ accumulation and microglial activation in an AD mouse model. " 01/01/2014 - "In the present study, we assessed the influence of MyD88 deficiency on the oral innate defense, particularly the expression of antimicrobial proteins in salivary glands and production of salivary basal immunoglobulins, in mice. "
5.	Immunoglobulins (Immunoglobulin)IBA 01/01/2014 - "In the present study, we assessed the influence of MyD88 deficiency on the oral innate defense, particularly the expression of antimicrobial proteins in salivary glands and production of salivary basal immunoglobulins, in mice. "
6.	Egtazic Acid (EGTA)IBA 04/01/2015 - "Here, mouse models of superficial epithelial injury (tissue paper blotting with EGTA treatment) and immunocompromise (MyD88 deficiency) were used to study the contribution of the T3SS transcriptional activator ExsA to epithelial traversal. "
7.	CytokinesIBA 06/01/2014 - "The 3-h but not 72-h induction of cytokine transcript was abrogated by MyD88 deficiency. " 11/01/2007 - "We demonstrate that whereas MyD88 deficiency markedly impaired secretion of IFN-alpha, production of the cytokine was largely independent of TLR9 signaling, in the liver. " 12/01/2017 - "Myeloid and dendritic cell MyD88 deficiency also did not markedly impair cytokine production during infection. " 03/01/2006 - "MyD88 deficiency results in tissue-specific changes in cytokine induction and inflammation in interleukin-18-independent mice infected with Borrelia burgdorferi." 04/01/2011 - "Unexpectedly, we found that the transcript level of one proinflammatory cytokine was increased in sMLA-treated cells by MyD88 deficiency to the higher level induced by synthetic diphosphoryl lipid A, which suggested MyD88 may paradoxically help restrain proinflammatory signaling by TRIF-biased sMLA. "
8.	Interleukin-6 (Interleukin 6)IBA 10/29/2010 - "Finally, TLR2 or MyD88 deficiency was associated with decreased IL-6 and type 1 IFN production as well as increased viral burden during short-term challenges with MHV-68. " 05/15/2004 - "Under these conditions, however, TLR2 or MyD88 deficiency significantly protected mice from lethality, concomitantly with decreased circulating levels of TNF-alpha and IL-6. " 01/01/2023 - "In wild-type periodontitis mice, NAC-S2 administration decreased the cemento-enamel-junction-alveolar bone crest (CEJ-ABC) distance and the relative mRNA expression of TNF, IL-6, and IL-1β, while such phenomena could not be observed in TLR4 deficiency or Myd88 deficiency mice. " 10/01/2007 - "We now show that MyD88 deficiency blocks the expansion and activation of DC in which Lyn is also normally expressed, and prevents the hypersecretion of proinflammatory cytokines IL-6 and IL-12 by Lyn-deficient DC. "
9.	Messenger RNA (mRNA)IBA 02/01/2015 - "MyD88 deficiency markedly inhibited RANKL expression after LPS stimulation and increased OPG messenger RNA (mRNA) production. " 01/01/2023 - "In wild-type periodontitis mice, NAC-S2 administration decreased the cemento-enamel-junction-alveolar bone crest (CEJ-ABC) distance and the relative mRNA expression of TNF, IL-6, and IL-1β, while such phenomena could not be observed in TLR4 deficiency or Myd88 deficiency mice. " 10/01/2014 - "Selective MyD88 deficiency either in bone marrow (BM)-derived or non-BM-derived cells attenuated hepatic up-regulation of inflammasome mRNA, caspase-1 activation and IL-1β protein production, but only BM-derived cell-specific MyD88-deficiency attenuated liver injury. " 08/01/2012 - "MyD88 deficiency worsened the severity of I/R injury, as assessed using the histological grading system, measuring luminal contents of hemoglobin (a marker of intestinal bleeding), and counting apoptotic epithelial cells, while it inhibited the increase in mRNA expression of TNF-α and KC. "
10.	Toll-Like Receptors (Toll-Like Receptor)IBA 10/08/2021 - "Finally, MyD88 deficiency suppresses skin lesion development in OTULINE-KO mice, suggesting that toll-like receptor and/or IL-1 signalling are involved in mediating skin inflammation. " 12/01/2011 - "MyD88 deficiency attenuates angiotensin II-induced abdominal aortic aneurysm formation independent of signaling through Toll-like receptors 2 and 4." 07/31/2011 - "In FADD(IEC-KO) mice, TNF deficiency ameliorated colon inflammation, whereas MYD88 deficiency and also elimination of the microbiota prevented colon inflammation, indicating that bacteria-mediated Toll-like-receptor signalling drives colitis by inducing the expression of TNF and other cytokines. "