Different gene expression profiles in iPSC-derived motor neurons from ALS8 patients with variable clinical courses suggest mitigating pathways for neurodegeneration.

Amyotrophic lateral sclerosis type 8 (ALS8) is an autosomal dominant form of ALS, which is caused by pathogenic variants in the VAPB gene. Here we investigated five ALS8 patients, classified as 'severe' and 'mild' from a gigantic Brazilian kindred, carrying the same VAPB mutation but displaying different clinical courses. Copy number variation and whole exome sequencing analyses in such individuals ruled out previously described genetic modifiers of pathogenicity.

ATLANTIC EPIPHYTES: a data set of vascular and non-vascular epiphyte plants and lichens from the Atlantic Forest.

Epiphytes are hyper-diverse and one of the frequently undervalued life forms in plant surveys and biodiversity inventories. Epiphytes of the Atlantic Forest, one of the most endangered ecosystems in the world, have high endemism and radiated recently in the Pliocene. We aimed to (1) compile an extensive Atlantic Forest data set on vascular, non-vascular plants (including hemiepiphytes), and lichen epiphyte species occurrence and abundance; (2) describe the epiphyte distribution in the Atlantic Forest, in order to indicate future sampling efforts.

The advertisement call of Bokermannohyla flavopicta Leite, Pezzuti & Garcia, 2012 (Anura: Hylidae) from the mountains of Chapada Diamantina, Bahia, Brazil.

Bokermannohyla Faivovich, Haddad, Garcia, Frost, Campbell & Wheeler (2005) is a Brazilian treefrog genus currently composed of 32 species (Brandão et al. 2012; Leite et al. 2012; Frost 2015).

A new species of Hatchet-faced Treefrog Sphaenorhynchus Tschudi (Anura: Hylidae) from Quadrilátero Ferrífero, Minas Gerais, southeastern Brazil.

A new species of Sphaenorhynchus is described from the Municipality of Mariana, State of Minas Gerais, Brazil. It is characterized by the vocal sac moderately developed, single, subgular, with longitudinal folds; white canthal and dorsolateral lines delimited below by a dorsolateral black line from the tip of snout extending beyond the eye to gradually disappearing up to the flanks; and premaxilla and maxilla almost completely edentulous, each bearing 1-5 extremely small teeth. It is most similar with Sphaenorhynchus orophilus, from which it can be distinguished by having a less robust forearm in males; glandular subcloacal dermal fold; premaxilla and maxilla almost completely edentulous; and larvae with large marginal papillae in the oral disc.

The tadpoles of two species of the Bokermannohyla circumdata group (Hylidae, Cophomantini).

We describe the external morphology and oral cavity of the tadpoles of Bokermannohyla caramaschii and B. diamantina respectively from the states of Espírito Santo and Bahia, Brazil. Larvae of both species are distinguished from each other by external characters such as body shape, labial tooth-row formula, number of marginal papillae, coloration and internal oral anatomy features.

Reduced passive force in skeletal muscles lacking protein arginylation.

Arginylation is a posttranslational modification that plays a global role in mammals. Mice lacking the enzyme arginyltransferase in skeletal muscles exhibit reduced contractile forces that have been linked to a reduction in myosin cross-bridge formation. The role of arginylation in passive skeletal myofibril forces has never been investigated.

Residual force depression in single sarcomeres is abolished by MgADP-induced activation.

The mechanisms behind the shortening-induced force depression commonly observed in skeletal muscles remain unclear, but have been associated with sarcomere length non-uniformity and/or crossbridge inhibition. The purpose of this study was twofold: (i) to evaluate if force depression is present in isolated single sarcomeres, a preparation that eliminates sarcomere length non-uniformities and (ii) to evaluate if force depression is inhibited when single sarcomeres are activated with MgADP, which biases crossbridges into a strongly-bound state. Single sarcomeres (n = 16) were isolated from rabbit psoas myofibrils using two micro-needles (one compliant, one rigid), piercing the sarcomere externally adjacent to the Z-lines.

Testing the relationship between human occupancy in the landscape and tadpole developmental stress.

Amphibian population declines are widespread; the main causal factors are human related and include habitat fragmentation due to agriculture, mining, fires, and urban development. Brazil is the richest country in species of amphibians, and the Brazilian regions with the greatest amphibian diversity are experiencing relatively high rates of habitat destruction, but there are presently relatively few reports of amphibian declines. It is thus important to develop research methods that will detect deterioration in population health before severe declines occur.

Non-crossbridge forces in activated striated muscles: a titin dependent mechanism of regulation?

When skeletal muscles are stretched during activation in the absence of myosin-actin interactions, the force increases significantly. The force remains elevated throughout the activation period. The mechanism behind this non-crossbridge force, referred to as static tension, is unknown and generates debate in the literature.

Effects of controlled mechanical ventilation on sepsis-induced diaphragm dysfunction in rats.

Objectives: Diaphragm dysfunction develops during severe sepsis as a consequence of hemodynamic, metabolic, and intrinsic abnormalities. Similarly, 12 hours of controlled mechanical ventilation also promotes diaphragm dysfunction. Importantly, patients with sepsis are often treated with mechanical ventilation for several days.

Arginylation of myosin heavy chain regulates skeletal muscle strength.

Protein arginylation is a posttranslational modification with an emerging global role in the regulation of actin cytoskeleton. To test the role of arginylation in the skeletal muscle, we generated a mouse model with Ate1 deletion driven by the skeletal muscle-specific creatine kinase (Ckmm) promoter. Ckmm-Ate1 mice were viable and outwardly normal; however, their skeletal muscle strength was significantly reduced in comparison to controls.

A new species of Crossodactylodes (Anura: Leptodactylidae) from Minas Gerais, Brazil: first record of genus within the Espinhaço Mountain Range.

The genus Crossodactylodes comprises three species of Atlantic Rainforest endemic frogs strictly dependent on bromeliads where they spend their entire life cycle. The current geographic distribution of the genus covers highland areas of Atlantic Rainforest in the States of Rio de Janeiro and Espírito Santo, Southeastern Brazil. We describe a new species of the genus from Parque Estadual do Pico do Itambé, at Santo Antônio do Itambé municipality, State of Minas Gerais, Southeastern Brazil.

A new species of the Scinax catharinae group (Anura, Hylidae) from Serra da Canastra, southwestern state of Minas Gerais, Brazil.

We describe Scinax pombali sp. n. a new species of treefrog of the Scinax catharinae group from Serra da Canastra, municipality of Capitólio (20°36'03''S, 46°17'34.

Peptidomic dissection of the skin secretion of Phasmahyla jandaia (Bokermann and Sazima, 1978) (Anura, Hylidae, Phyllomedusinae).

The systematic investigation of the peptidic composition of the skin secretion of Phasmahyla jandaia, a phyllomedusine anuran endemic to the southern region of the Espinhaço range in Brazil, is herein reported. By means of de novo interpretation of tandem mass spectrometric data, Edman N-terminal sequencing and similarity searches, 57 peptides - including phylloseptins, dermaseptins stricto sensu, dermatoxins, hyposins, tryptophyllins, caerulein-related, bradykinin-related, bradykinin potentiating, tyrosine-rich, and opioid peptides - were sequenced. Moreover, five peptide families without significant similarity to other known molecules were verified.