# data[].attributes.body
Por favor construam uma tabela, pode ser partilhada e coloquem aqui o link onde vão colocando o que estão a fazer e as vossas dúvidas. Coloquem tb se poderem uma foto de todos para eu me relembrar das caras . Têm que dividir as tarefas. :)obrigada

# data[].attributes.body
https://docs.google.com/document/d/1BJ8o7xY2MKZJzK_4GYonW7niia3n8weGw-eVBbeE994/edit

# data[].attributes.attachment_link.description
Create a new document and edit with others at the same time -- from your computer, phone or tablet. Get stuff done with or without an internet connection. Use Docs to edit Word files. Free from Google.

# data[].attributes.body
1. Introdução

2. Considerações gerais

2.1. Noções gerais de fisiologia pulmonar

2.2. Constituintes celulares do pulmão

2.2.1. Vias aéreas

2.2.2. Alvéolos

2.2.3. Endotélio capilar
3. Vias metabólicas preferenciais do pulmão

3.1. Metabolismo dos hidratos de carbono

3.2. Metabolismo de lípidos

3.2.1. Metabolismo do surfactante

3.3. Metabolismo de aminas biológicas, péptidos e prostaglandinas
4. Conclusão

# data[].attributes.body
https://docs.google.com/document/d/14fQkWxIP8WD8DRWwPNBCilWLyE_2sMwrpqB3u7BJOgw/edit?usp=sharing

# data[].attributes.body
Ana Rita Mendes
Diana Reis
Eduarda Coelho
Lara Robalo
Nicole Martins

# data[].attributes.body
Servier biomedical art
Biorender

# data[].attributes.body
https://docs.google.com/document/d/10ZWjD2Z1hWgsHABcip3ZSpDoLqqonBt6B7ivUVEK_Pc/edit

# data[].attributes.attachment_link.description
Create a new document and edit with others at the same time -- from your computer, phone or tablet. Get stuff done with or without an internet connection. Use Docs to edit Word files. Free from Google.

# data[].attributes.body
https://docs.google.com/document/d/1INs2PTEbtnJ7jYowienZcfPQAqlbHsM34u_pZ2U6feE/edit

# data[].attributes.attachment_link.description
Create a new document and edit with others at the same time -- from your computer, phone or tablet. Get stuff done with or without an internet connection. Use Docs to edit Word files. Free from Google.

# data[].attributes.body
https://pubmed.ncbi.nlm.nih.gov/28025703/

# data[].attributes.attachment_link.readable_text
Affiliations

¹ Pulmonary, Allergy, and Critical Care Division, Department of Medicine, Surfactant Biology Laboratories, University of Pennsylvania Perelman School of Medicine, Edward J. Stemmler Hall, Suite 218, 3450 Hamilton Walk, Philadelphia, PA 19104, USA.

² Pulmonary, Allergy, and Critical Care Division, Department of Medicine, Surfactant Biology Laboratories, University of Pennsylvania Perelman School of Medicine, Edward J. Stemmler Hall, Suite 218, 3450 Hamilton Walk, Philadelphia, PA 19104, USA. mulugeta@mail.med.upenn.edu.

Free PMC article

Free PMC article

Affiliations

¹ Pulmonary, Allergy, and Critical Care Division, Department of Medicine, Surfactant Biology Laboratories, University of Pennsylvania Perelman School of Medicine, Edward J. Stemmler Hall, Suite 218, 3450 Hamilton Walk, Philadelphia, PA 19104, USA.

² Pulmonary, Allergy, and Critical Care Division, Department of Medicine, Surfactant Biology Laboratories, University of Pennsylvania Perelman School of Medicine, Edward J. Stemmler Hall, Suite 218, 3450 Hamilton Walk, Philadelphia, PA 19104, USA. mulugeta@mail.med.upenn.edu.

Abstract

The lipid transporter, ATP-binding cassette class A3 (ABCA3), is a highly conserved multi-membrane-spanning protein that plays a critical role in the regulation of pulmonary surfactant homeostasis. Mutations in ABCA3 have been increasingly recognized as one of the causes of inherited pulmonary diseases. These monogenic disorders produce familial lung abnormalities with pathological presentations ranging from neonatal surfactant-deficiency-induced respiratory failure to childhood or adult diffuse parenchymal lung diseases for which specific treatment modalities remain limited. More than 200 ABCA3 mutations have been reported to date with approximately three quarters of patients presenting as compound heterozygotes. Recent advances in our understanding of the molecular basis underlying normal ABCA3 biosynthesis and processing and of the mechanisms of alveolar epithelial cell dysregulation caused by the expression of its mutant forms are beginning to emerge. These insights and the role of environmental factors and modifier genes are discussed in the context of the considerable variability in disease presentation observed in patients with identical ABCA3 gene mutations. Moreover, the opportunities afforded by an enhanced understanding of ABCA3 biology for targeted therapeutic strategies are addressed.

Keywords: ABCA3; Compound heterozygous mutations; Inhertited pulmonary disease; Pulmonary surfactant; Surfactant deficiency.

Similar articles

Ultrastructural characterization of genetic diffuse lung diseases in infants and children: a cohort study and review.

Citti A, Peca D, Petrini S, Cutrera R, Biban P, Haass C, Boldrini R, Danhaive O. Citti A, et al. Ultrastruct Pathol. 2013 Oct;37(5):356-65. doi: 10.3109/01913123.2013.811454. Ultrastruct Pathol. 2013. PMID: 24047351

Ultrastructural and molecular analysis in fatal neonatal interstitial pneumonia caused by a novel ABCA3 mutation.

Bruder E, Hofmeister J, Aslanidis C, Hammer J, Bubendorf L, Schmitz G, Rufle A, Bührer C. Bruder E, et al. Mod Pathol. 2007 Oct;20(10):1009-18. doi: 10.1038/modpathol.3800928. Epub 2007 Jul 27. Mod Pathol. 2007. PMID: 17660803

Extracellular Lipids in the Lung and Their Role in Pulmonary Fibrosis.

Burgy O, Loriod S, Beltramo G, Bonniaud P. Burgy O, et al. Cells. 2022 Apr 3;11(7):1209. doi: 10.3390/cells11071209. Cells. 2022. PMID: 35406772 Free PMC article. Review.

Gene Therapy Potential for Genetic Disorders of Surfactant Dysfunction.

Cooney AL, Wambach JA, Sinn PL, McCray PB Jr. Cooney AL, et al. Front Genome Ed. 2022 Jan 14;3:785829. doi: 10.3389/fgeed.2021.785829. eCollection 2021. Front Genome Ed. 2022. PMID: 35098209 Free PMC article. Review.

Channels and Transporters of the Pulmonary Lamellar Body in Health and Disease.

Dietl P, Frick M. Dietl P, et al. Cells. 2021 Dec 24;11(1):45. doi: 10.3390/cells11010045. Cells. 2021. PMID: 35011607 Free PMC article. Review.

# data[].attributes.attachment_link.keywords[]
atp-binding cassette transporters / chemistry

# data[].attributes.attachment_link.keywords[]
atp-binding cassette transporters / genetics

# data[].attributes.attachment_link.keywords[]
atp-binding cassette transporters / metabolism*

# data[].attributes.attachment_link.keywords[]
national center for biotechnology information

# data[].attributes.attachment_link.title
The biology of the ABCA3 lipid transporter in lung health and disease - PubMed

# data[].attributes.attachment_link.description
The lipid transporter, ATP-binding cassette class A3 (ABCA3), is a highly conserved multi-membrane-spanning protein that plays a critical role in the regulation of pulmonary surfactant homeostasis. Mutations in ABCA3 have been increasingly recognized as one of the causes of inherited pulmonary disea ...

# data[].attributes.body
O nosso grupo já está inscrito!

# data[].attributes.attachment_link.title
Poster grupo 7  última versão corrigida 21-04

# data[].attributes.body
Já submetemos o poster no concurso!

# data[].attributes.body
1. D
2. A