Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects to both the upper and lower motor neuron. We reported a rare presentation of ALS with SPG11 mutation with heterozygous state, contrary to the classic autosomal recessive form of ALS associated with this mutation, thus documenting the third case found with probable association with said pattern of inheritance, and the first of related ALS a mutation of SPG11 in Mexico. This allows us to reaffirm the genetic heterogeneity of ALS and the prognostic importance of the determination of such rare mutations with less lethal course to the classical form.

Resumen El presente artículo tiene como objetivo identificar el/los propósito(s) actual(es) de los catálogos de autoridad de nombres personales, a partir de los metadatos (elementos/atributos) que han sido incluidos en dichos catálogos, tras la aplicación de estándares de normalización. Para lograr este objetivo, se recurrió al análisis documental como método de investigación, de modo que se hizo una revisión y un análisis de la literatura existente en torno al control de autoridad, los catálogos de autoridad y los estándares de normalización empleados en el desarrollo de dichos catálogos. El análisis de la información localizada en la literatura se hizo bajo un enfoque cualitativo con alcance descriptivo. Se identificó que el control de autoridad sobre nombres personales y su realización mediante la aplicación de estándares como las GARE, las GARR y AACR, ha tenido y tiene un propósito organizativo, mismo que se ve reflejado en la elaboración de catálogos de autoridad. Sin embargo, con la introducción de estándares como FRAD, RDA y LRM, el propósito de dichos catálogos puso énfasis en el aspecto informativo. Es así que esta investigación sostiene que en la actualidad el propósito de los catálogos de autoridad de nombres personales, además de ser organizativo, es -o puede ser- informativo, dependiendo de los metadatos (elementos/atributos) que sean incluidos.

Abstract This article aims to identify the current purpose(s) of personal name authority catalogues, based on the metadata (elements/attributes) that have been included in said catalogues, after the application of standards of normalization. To achieve the objective, documentary analysis was used as a research method, so that a review and analysis of the existing literature was made around authority control, authority catalogs and normalization standards used in the development of these catalogs. The analysis of the information located in the literature was done under a qualitative approach with a descriptive scope. It was identified that the control of authority over personal names, and its realization through the application of standards such as the GARE, the GARR and AACR, has had and has an organizational purpose, which is reflected in the elaboration of catalogs of authority. However, with the introduction of standards such as FRAD, RDA and LRM, the purpose of such catalogs put an emphasis on informational aspect. Thus, this research maintains that the current purpose of personal name authority catalogues, in addition to being organizational, is/can be informative, depending on the metadata (elements/ attributes) that are included.

ABSTRACT The objective of this study was to evaluate the effects of four levels of fish oil on lamb performance, carcass yield, ruminal fermentation, and leptin gene expression in perirenal fat. Thirty-two lambs (24.10±2.15 kg, Katahdin × Pelibuey) were used in a completely randomized experimental design. The lambs were assigned to one of four dietary treatments (n = 8 lambs/treatment), expressed as g/kg DM basis: 0 fish oil and 300 corn; 10 fish oil and 250 corn; 20 fish oil and 205 corn; and 30 fish oil and 170 corn. The lambs were weighed on consecutive days at the beginning (days 0 and 1) and at the end (days 55 and 56) of the trial. Ruminal fluid samples were collected on day 56 to evaluate the ruminal fermentation pattern. The lambs were slaughtered on day 56; perirenal adipose tissue samples were collected and the carcass yields were recorded. Volatile fatty acids, ammonia N, and leptin mRNA expression were not affected by the dietary treatments. However, the dry matter intake, average daily gain, final body weight, and the hot carcass yield showed either increased linear or quadratic responses as the proportion of fish oil increased in the ration; the estimated optimal level obtained of fish oil levels for average daily gain was 11.2±0.21 g/kg and 12.8±4.67 g/kg for feed conversion. Additionally, feed efficiency and backfat thickness had an increment, showing quadratic response as the proportion of fish oil increased in the diet. Increasing the fish oil concentration in the diet does not affect leptin messenger ribonucleic acid expression. The lamb performance can be improved with 12 g/kg fish oil in diets of finishing lambs.