Disease: Cutis laxa- recessive
- <em>FBLN5</em>-Related Cutis Laxa Syndrome: A Case with a Novel Variant and Review of the Literature
- A 5-year Journey with Cutis Laxa in an Indian Child: The De Barsy Syndrome Revisited
- A de novo 1q23.3-q24.2 deletion combined with a GORAB missense mutation causes a distinctive phenotype with cutis laxa
- A Novel <em>ATP6V0A2</em> Mutation Causing Recessive Cutis Laxa with Unusual Manifestations of Bleeding Diathesis and Defective Wound Healing
- A Novel ATP6V0A2 Mutation Causing Recessive Cutis Laxa with Unusual Manifestations of Bleeding Diathesis and Defective Wound Healing
- A novel deletion mutation in the ATP6V0A2 gene in an Iranian patient affected by autosomal recessive cutis laxa
- A novel elastin gene frameshift mutation in a Russian family with cutis laxa: a case report
- A proposal of rehabilitative approach in the rare disease "De Barsy Syndrome": case report
- A Transcriptome Study of Progeroid Neurocutaneous Syndrome Reveals POSTN As a New Element in Proline Metabolic Disorder
- Analysis of clinical features and genetic variants in a child with autosomal recessive cutis laxa due to variants of ATP6V0A2 gene
- Ascending Aortic Aneurysm in a Child With Fibulin-4 Deficiency
- ATP6V0A2 mutations present in two Mexican Mestizo children with an autosomal recessive cutis laxa syndrome type IIA
- ATP6V1A variants are associated with childhood epilepsy with favorable outcome
- ATP7A mutation with occipital horns and distal motor neuropathy: A continuum
- Autosomal Recessive Cutis Laxa 1C Mutations Disrupt the Structure and Interactions of Latent TGFbeta Binding Protein-4
- Autosomal Recessive Cutis Laxa 1C Mutations Disrupt the Structure and Interactions of Latent TGFβ Binding Protein-4
- Autosomal recessive cutis laxa type 1C with a homozygous LTBP4 splicing variant: a case report and update of literature
- Autosomal recessive cutis laxa type Ib-Successful redo aortic root and arch replacement
- Autosomal Recessive Cutis Laxa Type II: Report of Novel Mutation in a Child
- Autosomal recessive cutis laxa type IIIA: Report of a patient with severe phenotype and review of the literature
- Autosomal recessive cutis laxa: a novel mutation in the FBLN5 gene in a family
- Autosomal recessive cutis laxa: New genes identified
- B3GAT3-related linkeropathy and an in-frame homozygous deletion in an adult patient
- Bi-allelic premature truncating variants in LTBP1 cause cutis laxa syndrome
- Case Report: Occurrence of Severe Thoracic Aortic Aneurysms (Involving the Ascending, Arch, and Descending Segments) as a Result of Fibulin-4 Deficiency: A Rare Pathology With Successful Management
- Clinical and genetic analysis of a patient with cutis laxa
- Clinical and molecular characterization of a 13-year-old Indian boy with cutis laxa type 2B: Identification of two novel PYCR1 mutations by amplicon-based semiconductor exome sequencing
- Clinical and molecular characterization of an 18-month-old infant with autosomal recessive cutis laxa type 1C due to a novel LTBP4 pathogenic variant, and literature review
- Clinical implications of de Barsy syndrome
- Clinical presentation of a patient with cutis laxa with systemic involvement: a case report
- Compound heterozygous mutations in two different domains of ALDH18A1 do not affect the amino acid levels in a patient with hereditary spastic paraplegia
- Confirming the enzymatic activity and neurodevelopmental trajectory of PYCR1 mutation in one child with autosomal-recessive cutis laxa type 2
- Congenital Cutis Laxa Type 2 Associated With Recurrent Aspiration Pneumonia and Growth Delay: Case Report
- Congenital cutis laxa: a case study
- Consanguinity and Double Recessive Gene Pathology: Cutis Laxa (PYCR1) and Nephrotic Syndrome (PLCE1)
- Corkscrew Mesenteric Arteries and Tortuous Descending Aorta in Autosomal Recessive Cutis Laxa
- Cutis Laxa
- Cutis Laxa
- Cutis laxa autosomal recessive type II or wrinkly skin syndrome?
- Cutis laxa presenting as recurrent ileus
- Cutis Laxa syndrome: a case report
- Cutis laxa with pulmonary emphysema, conjunctivochalasis, nasolacrimal duct obstruction, abnormal hair, and a novel FBLN5 mutation
- De Barsy Syndrome: A Case Report of a Rare Genetic Disorder
- Discriminative Features in Three Autosomal Recessive Cutis Laxa Syndromes: Cutis Laxa IIA, Cutis Laxa IIB, and Geroderma Osteoplastica
- Effect of R119G Mutation on Human P5CR1 Dynamic Property and Enzymatic Activity
- EMILIN1 deficiency causes arterial tortuosity with osteopenia and connects impaired elastogenesis with defective collagen fibrillogenesis
- Expanding the clinical and genetic heterogeneity of hereditary disorders of connective tissue
- Expanding the clinical and molecular spectrum of ATP6V1A related metabolic cutis laxa
- Expanding the phenotypic spectrum of ALDH18A1-related autosomal recessive cutis laxa with a description of novel neuroradiological findings
- Expanding the Spectrum of Neurological Manifestations in Cutis Laxa, Autosomal Recessive, Type IIIA
- Extending the ALDH18A1 clinical spectrum to severe autosomal recessive fetal cutis laxa with corpus callosum agenesis
- FAM160B1 deficit associated with microcephaly, severe intellectual disability, ataxia, behavioral abnormalities and speech problems
- FBLN5-Related Cutis Laxa Syndrome: A Case with a Novel Variant and Review of the Literature
- Fibulin-4 E57K Knock-in Mice Recapitulate Cutaneous, Vascular and Skeletal Defects of Recessive Cutis Laxa 1B with both Elastic Fiber and Collagen Fibril Abnormalities
- Fibulin-4 is essential for maintaining arterial wall integrity in conduit but not muscular arteries
- First report of a short in-frame biallelic deletion removing part of the EGF-like domain calcium-binding motif in LTBP4 and causing autosomal recessive cutis laxa type 1C
- Function of Ltbp-4L and fibulin-4 in survival and elastogenesis in mice
- Genetic analysis of Pycr1 and Pycr2 in mice
- Giant aortic aneurysm due to fibulin- 4 deficiency: case series
- Giant ascending aortic aneurysm with impending rupture as presentation of cutis laxa 1B: a case report
- Hemopericardium with cardiac tamponade as a rare presentation of a massive aortic aneurysm in a young child with autosomal recessive cutis laxa
- Homozygous deletion of MYADML2 in cranial asymmetry, reduced bone maturation, multiple dislocations, lumbar lordosis, and prominent clavicles
- Identification of a de novo mutation of the elastin gene by targeted exome sequencing in autosomal dominant cutis laxa
- Identification of a Novel 19-bp Deletion Mutation in <em>LTBP4</em> Using Exome Sequencing in Two Siblings with Autosomal Recessive Cutis Laxa Type 1C
- Identification of a Novel 19-bp Deletion Mutation in LTBP4 Using Exome Sequencing in Two Siblings with Autosomal Recessive Cutis Laxa Type 1C
- Keratoglobus with ARCL1B (EFEMP2 gene) cutis laxa
- Latent transforming growth factor binding protein 4 regulates transforming growth factor beta receptor stability
- Leukoencephalopathy in RIN2 syndrome: Novel mutation and expansion of clinical spectrum
- Loss of ALDH18A1 function is associated with a cellular lipid droplet phenotype suggesting a link between autosomal recessive cutis laxa type 3A and Warburg Micro syndrome
- Loss of fibulin-4 disrupts collagen synthesis and maturation: implications for pathology resulting from EFEMP2 mutations
- Loss of zebrafish atp6v1e1b, encoding a subunit of vacuolar ATPase, recapitulates human ARCL type 2C syndrome and identifies multiple pathobiological signatures
- LTBP4 in Health and Disease
- Modeling autosomal recessive cutis laxa type 1C in mice reveals distinct functions for Ltbp-4 isoforms
- Multiple bladder diverticula caused by occipital horn syndrome
- Mutations in ATP6V1E1 or ATP6V1A Cause Autosomal-Recessive Cutis Laxa
- Mutations in ATP6V1E1 or ATP6V1A Cause Autosomal-Recessive Cutis Laxa
- Mutations in Either TUBB or MAPRE2 Cause Circumferential Skin Creases Kunze Type
- N-Acetylcysteine Therapy in an Infant with Transaldolase Deficiency Is Well Tolerated and Associated with Normalization of Alpha Fetoprotein Levels
- New insight into clinical heterogeneity and inheritance diversity of FBLN5-related cutis laxa
- Novel compound heterozygous mutations identified by whole exome sequencing in a Japanese patient with geroderma osteodysplastica
- NOVEL RETINAL FINDINGS IN A PATIENT WITH AUTOSOMAL RECESSIVE CUTIS LAXA TYPE 2A
- Overview of the Pulmonary Manifestations in Patients with Autosomal Recessive Cutis Laxa Type IC
- Pathological Fracture in Autosomal Recessive Cutis Laxa Type 2B
- Polymicrogyria and myoclonic epilepsy in autosomal recessive cutis laxa type 2A
- Progeroid syndrome of De Barsy - a case report and review of ophthalmic literature
- Recurrent De Novo Mutations Affecting Residue Arg138 of Pyrroline-5-Carboxylate Synthase Cause a Progeroid Form of Autosomal-Dominant Cutis Laxa
- Review of clinical and molecular variability in autosomal recessive cutis laxa 2A
- RIN2 syndrome: Expanding the clinical phenotype
- Severe congenital cutis laxa: Identification of novel homozygous LOX gene variants in two families
- Severe Phenotype of Cutis Laxa Type 1B with Antenatal Signs due to a Novel Homozygous Nonsense Mutation in <em>EFEMP2</em>
- Severe Phenotype of Cutis Laxa Type 1B with Antenatal Signs due to a Novel Homozygous Nonsense Mutation in EFEMP2
- SOPH syndrome in three affected individuals showing similarities with progeroid cutis laxa conditions in early infancy
- SPG9A with the new occurrence of an ALDH18A1 mutation in a CMT1A family with PMP22 duplication: case report
- The Diagnostic Dilemma of Cutis Laxa: A Report of Two Cases with Genotypic Dissimilarity
- The Role of Cardiovascular Surgery in the Management of a Patient Diagnosed With Congenital Cutis Laxa Syndrome Complicated by Multivalvular Heart Disease
- Tremor as an early sign of hereditary spastic paraplegia due to mutations in ALDH18A1
- Two fetuses in one family of arterial tortuosity syndrome: prenatal ultrasound diagnosis
- Two novel compound heterozygous variants of LTBP4 in a Chinese infant with cutis laxa type IC and a review of the related literature
- Two novel homozygous variants of ATP6V0A2 and ALDH18A1 lead to autosomal recessive cutis laxa type 2 and 3 in two Pakistani families
- Δ<sup>1</sup> -Pyrroline-5-carboxylate synthetase deficiency: An emergent multifaceted urea cycle-related disorder