TY  - JOUR
A1  - Lim, Sze Chern
A1  - Friemel, Martin
A1  - Marum, Justine E.
A1  - Tucker, Elena J.
A1  - Bruno, Damien L.
A1  - Riley, Lisa G.
A1  - Christodoulou, John
A1  - Kirk, Edwin P.
A1  - Boneh, Avihu
A1  - DeGennaro, Christine M.
A1  - Springer, Michael
A1  - Mootha, Vamsi K.
A1  - Rouault, Tracey A.
A1  - Leimkühler, Silke
A1  - Thorburn, David R.
A1  - Compton, Alison G.
T1  - Mutations in LYRM4, encoding ironsulfur cluster biogenesis factor ISD11, cause deficiency of multiple respiratory chain complexes
JF  - Human molecular genetics
N2  - Ironsulfur clusters (ISCs) are important prosthetic groups that define the functions of many proteins. Proteins with ISCs (called ironsulfur or FeS proteins) are present in mitochondria, the cytosol, the endoplasmic reticulum and the nucleus. They participate in various biological pathways including oxidative phosphorylation (OXPHOS), the citric acid cycle, iron homeostasis, heme biosynthesis and DNA repair. Here, we report a homozygous mutation in LYRM4 in two patients with combined OXPHOS deficiency. LYRM4 encodes the ISD11 protein, which forms a complex with, and stabilizes, the sulfur donor NFS1. The homozygous mutation (c.203GT, p.R68L) was identified via massively parallel sequencing of 1000 mitochondrial genes (MitoExome sequencing) in a patient with deficiency of complexes I, II and III in muscle and liver. These three complexes contain ISCs. Sanger sequencing identified the same mutation in his similarly affected cousin, who had a more severe phenotype and died while a neonate. Complex IV was also deficient in her skeletal muscle. Several other FeS proteins were also affected in both patients, including the aconitases and ferrochelatase. Mutant ISD11 only partially complemented for an ISD11 deletion in yeast. Our in vitro studies showed that the l-cysteine desulfurase activity of NFS1 was barely present when co-expressed with mutant ISD11. Our findings are consistent with a defect in the early step of ISC assembly affecting a broad variety of FeS proteins. The differences in biochemical and clinical features between the two patients may relate to limited availability of cysteine in the newborn period and suggest a potential approach to therapy.
Y1  - 2013
U6  - https://doi.org/10.1093/hmg/ddt295
SN  - 0964-6906
SN  - 1460-2083
VL  - 22
IS  - 22
SP  - 4460
EP  - 4473
PB  - Oxford Univ. Press
CY  - Oxford
ER  - 
TY  - JOUR
A1  - Falk, Thomas
A1  - Kirk, Michael
A1  - Lohmann, Dirk
A1  - Kruger, Bertus
A1  - Hüttich, Christian
A1  - Kamukuenjandje, Richard
T1  - The profits of excludability and transferability in redistributive land reform in central Namibia
JF  - Development Southern Africa
N2  - Policies which redistribute property rights to land can improve the well-being of rural households and can have overall growth effects. In many cases, however, land reforms are driven mainly by politically justified objectives. Under such circumstances, little emphasis is placed on whether and, if so, how property rights can increase productivity. Following 18 years of land reform implementation in Namibia, we evaluated 65 beneficiaries in Namibia. We assess to which degree land rights affects their farm income. The study focuses on Namibia’s two main commercial land reform instruments, namely the Farm Unit Resettlement Scheme and the Affirmative Action Loan Scheme. We find evidence that the majority of land reform projects are not profitable. Further, our study confirms the importance of the right to restrict land access compared with the right to transfer. The long-term leasehold contract seemingly provides sufficient incentives to make productive use of the land.
KW  - Redistributive land reform
KW  - property rights
KW  - farm productivity
KW  - pastoralism
KW  - Namibia
Y1  - 2017
U6  - https://doi.org/10.1080/0376835X.2016.1269633
SN  - 0376-835X
SN  - 1470-3637
VL  - 34
SP  - 314
EP  - 329
PB  - Routledge, Taylor & Francis Group
CY  - Abingdon
ER  - 
TY  - JOUR
A1  - Noonan, Michael J.
A1  - Tucker, Marlee A.
A1  - Fleming, Christen H.
A1  - Akre, Thomas S.
A1  - Alberts, Susan C.
A1  - Ali, Abdullahi H.
A1  - Altmann, Jeanne
A1  - Antunes, Pamela Castro
A1  - Belant, Jerrold L.
A1  - Beyer, Dean
A1  - Blaum, Niels
A1  - Boehning-Gaese, Katrin
A1  - Cullen Jr, Laury
A1  - de Paula, Rogerio Cunha
A1  - Dekker, Jasja
A1  - Drescher-Lehman, Jonathan
A1  - Farwig, Nina
A1  - Fichtel, Claudia
A1  - Fischer, Christina
A1  - Ford, Adam T.
A1  - Goheen, Jacob R.
A1  - Janssen, Rene
A1  - Jeltsch, Florian
A1  - Kauffman, Matthew
A1  - Kappeler, Peter M.
A1  - Koch, Flavia
A1  - LaPoint, Scott
A1  - Markham, A. Catherine
A1  - Medici, Emilia Patricia
A1  - Morato, Ronaldo G.
A1  - Nathan, Ran
A1  - Oliveira-Santos, Luiz Gustavo R.
A1  - Olson, Kirk A.
A1  - Patterson, Bruce D.
A1  - Paviolo, Agustin
A1  - Ramalho, Emiliano Estero
A1  - Rosner, Sascha
A1  - Schabo, Dana G.
A1  - Selva, Nuria
A1  - Sergiel, Agnieszka
A1  - da Silva, Marina Xavier
A1  - Spiegel, Orr
A1  - Thompson, Peter
A1  - Ullmann, Wiebke
A1  - Zieba, Filip
A1  - Zwijacz-Kozica, Tomasz
A1  - Fagan, William F.
A1  - Mueller, Thomas
A1  - Calabrese, Justin M.
T1  - A comprehensive analysis of autocorrelation and bias in home range estimation
JF  - Ecological monographs : a publication of the Ecological Society of America.
N2  - Home range estimation is routine practice in ecological research. While advances in animal tracking technology have increased our capacity to collect data to support home range analysis, these same advances have also resulted in increasingly autocorrelated data. Consequently, the question of which home range estimator to use on modern, highly autocorrelated tracking data remains open. This question is particularly relevant given that most estimators assume independently sampled data. Here, we provide a comprehensive evaluation of the effects of autocorrelation on home range estimation. We base our study on an extensive data set of GPS locations from 369 individuals representing 27 species distributed across five continents. We first assemble a broad array of home range estimators, including Kernel Density Estimation (KDE) with four bandwidth optimizers (Gaussian reference function, autocorrelated‐Gaussian reference function [AKDE], Silverman's rule of thumb, and least squares cross‐validation), Minimum Convex Polygon, and Local Convex Hull methods. Notably, all of these estimators except AKDE assume independent and identically distributed (IID) data. We then employ half‐sample cross‐validation to objectively quantify estimator performance, and the recently introduced effective sample size for home range area estimation ( N̂ area
) to quantify the information content of each data set. We found that AKDE 95% area estimates were larger than conventional IID‐based estimates by a mean factor of 2. The median number of cross‐validated locations included in the hold‐out sets by AKDE 95% (or 50%) estimates was 95.3% (or 50.1%), confirming the larger AKDE ranges were appropriately selective at the specified quantile. Conversely, conventional estimates exhibited negative bias that increased with decreasing N̂ area. To contextualize our empirical results, we performed a detailed simulation study to tease apart how sampling frequency, sampling duration, and the focal animal's movement conspire to affect range estimates. Paralleling our empirical results, the simulation study demonstrated that AKDE was generally more accurate than conventional methods, particularly for small N̂ area. While 72% of the 369 empirical data sets had >1,000 total observations, only 4% had an N̂ area >1,000, where 30% had an N̂ area <30. In this frequently encountered scenario of small N̂ area, AKDE was the only estimator capable of producing an accurate home range estimate on autocorrelated data.
KW  - animal movement
KW  - kernel density estimation
KW  - local convex hull
KW  - minimum convex polygon
KW  - range distribution
KW  - space use
KW  - telemetry
KW  - tracking data
Y1  - 2018
U6  - https://doi.org/10.1002/ecm.1344
SN  - 0012-9615
SN  - 1557-7015
VL  - 89
IS  - 2
PB  - Wiley
CY  - Hoboken
ER  -