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JOURNAL ARTICLE
Sushmita Singh, Amrit Lal Singh, Kamal Krishna Pal, Kiran K Reddy, K Gangadhara, Rinku Dey, M K Mahatma, Aman Verma, Narendra Kumar, C B Patel, Lokesh Kumar Thawait, Suhail Ahmed, Radha Navapara, Kirti Rani, Praveen Kona
UNLABELLED: Peanut is mostly grown in calcareous soils with high pH which are deficient in available iron (Fe2+ ) for plant uptake causing iron deficiency chlorosis (IDC). The most pertinent solution is to identify efficient genotypes showing tolerance to limited Fe availability in the soil. A field screening of 40 advanced breeding lines of peanut using NRCG 7472 and ICGV 86031 as IDC susceptible and tolerant checks, respectively, was envisaged for four years. PBS 22040 and 29,192 exhibited maximum tolerance while PBS 12215 and 12,185 were most susceptible...
May 2023: Physiology and Molecular Biology of Plants: An International Journal of Functional Plant Biology
#22
JOURNAL ARTICLE
Babur Ahmed, Simon Shaw, Oliver Pratt, Claire Forde, Simon Lal, Gordon Carlson Cbe
BACKGROUND: Parenteral nutrition (PN) deficient in mitochondrial substrates and thiamine may lead to acidosis. This, combined with fatigue seen in patients with intestinal failure (IF), may suggest suboptimal oxidative metabolism. We therefore studied oxygen utilisation in otherwise apparently well-nourished individuals with intestinal failure receiving long term PN. METHODS: This was a retrospective analysis conducted in a tertiary IF institution, from 2010 to 2019, comparing treadmill/bicycle cardiopulmonary exercise test (CPET) derived variables including peak oxygen consumption (VO2 peak ), anaerobic threshold (AT) and ventilatory efficiency (minute ventilation (VE)/CO2 output (VCO2 ) of patients with IF (cases) to those without (controls), matched in a 1:2 ratio for age ( ± 3 years), gender, use of beta-blockers and physiology parameters of p-POSSUM score ( ± 5)...
August 2023: Clinical Nutrition ESPEN
#23
JOURNAL ARTICLE
Jinmin Zhang, Yu Yu, Terry Dang, Kevin Lal, Hongli Wu
Glutaredoxins (Grx1 and Grx2) are thiol-repair antioxidant enzymes that play vital roles in cellular redox homeostasis and various cellular processes. This study aims to evaluate the functions of the glutaredoxin (Grx) system, including glutaredoxin 1 (Grx1) and glutaredoxin 2 (Grx2), using Grx1/Grx2 double knockout (DKO) mice as a model. We isolated primary lens epithelial cells (LECs) from wild-type (WT) and DKO mice for a series of in vitro analyses. Our results revealed that Grx1/Grx2 DKO LECs exhibited slower growth rates, reduced proliferation, and aberrant cell cycle distribution compared to WT cells...
June 3, 2023: Experimental Eye Research
#24
JOURNAL ARTICLE
Manisha Balwani, William Balistreri, Lorenzo D'Antiga, Jennifer Evans, Emilio Ros, Florian Abel, Don P Wilson
BACKGROUND AND AIMS: Lysosomal acid lipase deficiency (LAL-D) is a rare, autosomal recessive disease involving lysosomal accumulation of cholesteryl esters and triglycerides. The International Lysosomal Acid Lipase Deficiency Registry (NCT01633489), established in 2013 to understand LAL-D natural history and long-term outcomes, is accessible to centres caring for patients diagnosed by deficient LAL activity and/or biallelic pathogenic LIPA variants. We describe the registry population enrolled through 2 May 2022...
July 2023: Liver International: Official Journal of the International Association for the Study of the Liver
#25
JOURNAL ARTICLE
Valentina Bianco, Melanie Korbelius, Nemanja Vujic, Alena Akhmetshina, Melina Amor, Dagmar Kolb, Anita Pirchheim, Ivan Bradic, Katharina B Kuentzel, Martin Buerger, Silvia Schauer, Huyen T T Phan, Dominik Bulfon, Gerald Hoefler, Robert Zimmermann, Dagmar Kratky
OBJECTIVE: To date, the only enzyme known to be responsible for the hydrolysis of cholesteryl esters and triacylglycerols in the lysosome at acidic pH is lysosomal acid lipase (LAL). Lipid malabsorption in the small intestine (SI), accompanied by macrophage infiltration, is one of the most common pathological features of LAL deficiency. However, the exact role of LAL in intestinal lipid metabolism is still unknown. METHODS: We collected three parts of the SI (duodenum, jejunum, ileum) from mice with a global (LAL KO) or intestine-specific deletion of LAL (iLAL KO) and corresponding controls...
July 2023: Molecular Metabolism
#26
David A Suarez-Zamora, Maria M Rojas-Rojas, Felipe Ordoñez-Guerrero, Jacqueline Mugnier-Quijano, Rocio Lopez-Panqueva
Lysosomal acid lipase (LAL) deficiency is a rare, autosomal recessive disease caused by mutations in the LIPA gene, which produces cholesteryl ester and triglyceride accumulation predominantly in hepatocytes, adrenal glands, and gastrointestinal tract. We describe two new cases occurring in siblings, aged 5 and 7 years, who presented with hepatomegaly, dyslipidemia, and abnormal liver function. Percutaneous liver biopsy revealed portal inflammation, hypertrophic Kupffer cells with a foamy appearance and microvesicular steatosis with fibrosis...
2023: Revista Española de Patología
#27
REVIEW
Melanie Korbelius, Katharina B Kuentzel, Ivan Bradić, Nemanja Vujić, Dagmar Kratky
Lysosomal acid lipase (LAL) is the sole enzyme known to degrade neutral lipids in the lysosome. Mutations in the LAL-encoding LIPA gene lead to rare lysosomal lipid storage disorders with complete or partial absence of LAL activity. This review discusses the consequences of defective LAL-mediated lipid hydrolysis on cellular lipid homeostasis, epidemiology, and clinical presentation. Early detection of LAL deficiency (LAL-D) is essential for disease management and survival. LAL-D must be considered in patients with dyslipidemia and elevated aminotransferase concentrations of unknown etiology...
June 2023: Trends in Molecular Medicine
#28
JOURNAL ARTICLE
Xiangling Wang, Baiquan Song, Zhenzhen Wu, Xiaoyu Zhao, Xin Song, Muhammad Faheem Adil, Muhammad Riaz, Milan Kumar Lal, Wengong Huang
Boron (B) deficiency and consequent limitation of plant yield and quality, particularly of sugar beet (Beta vulgaris L.) has emerged as a maior problem,which is exacerbating due to cultivar dependent variability in B deficiency tolerance. Pertinently, the current study was designed to elucidate the physiological and molecular mechanisms of B deficiency tolerance of sugar beet varieties KWS1197 (B-efficient variety) and KWS0143 (B-inefficient variety). A hydroponic experiment was conducted employing two B levels B0...
March 8, 2023: Plant Physiology and Biochemistry: PPB
#29
JOURNAL ARTICLE
Ting Zhao, Sheng Liu, Nasser H Hanna, Shadia Jalal, Xinchun Ding, Jun Wan, Cong Yan, Hong Du
BACKGROUND: Myeloid-derived suppressor cells (MDSCs) are a heterogeneous population of cells in tumor microenvironment, which suppress antitumor immunity. Expansion of various MDSC subpopulations is closely associated with poor clinical outcomes in cancer. Lysosomal acid lipase (LAL) is a key enzyme in the metabolic pathway of neutral lipids, whose deficiency (LAL-D) in mice induces the differentiation of myeloid lineage cells into MDSCs. These Lal -/- MDSCs not only suppress immune surveillance but also stimulate cancer cell proliferation and invasion...
March 2023: Journal for Immunotherapy of Cancer
#30
REVIEW
Vikas Mangal, Milan Kumar Lal, Rahul Kumar Tiwari, Muhammad Ahsan Altaf, Salej Sood, Vijay Gahlaut, Ashish Bhatt, Ajay Kumar Thakur, Ravinder Kumar, Vinay Bhardwaj, Vinod Kumar, Brajesh Singh, Rajender Singh, Devendra Kumar
Abiotic stresses adversely affect the productivity and production of vegetable crops. The increasing number of crop genomes that have been sequenced or re-sequenced provides a set of computationally anticipated abiotic stress-related responsive genes on which further research may be focused. Knowledge of omics approaches and other advanced molecular tools have all been employed to understand the complex biology of these abiotic stresses. A vegetable can be defined as any component of a plant that is eaten for food...
March 13, 2023: Planta
#31
JOURNAL ARTICLE
Xiao Shuang, Wang Zhenming, Mei Zhu, Sun Si, Li Zuo
BACKGROUND: Thalassemia trait (TT) and iron deficiency anemia (IDA) are the most common conditions of microcytic hypochromic anemia (MHA) in pregnant women. Accurate discrimination between TT and IDA is an important issue, and better methods are urgently needed. Although considerable RBC formulas and indices have been developed since 1973, distinguishing between IDA and TT is still a challenging problem due to the diversity of various anemic populations. To address this problem, we assessed the diagnostic function of 43 different differential formulas in patients with microcytic anemia by using accuracy measures and recommending a new log-based differential formula...
February 8, 2023: BMC Pregnancy and Childbirth
#32
JOURNAL ARTICLE
Karthick Vasudevan, S Udhaya Kumar, A Mithun, B Raghavendra, C George Priya Doss
Wolman disorder (WD) was first described in Iranian-Jewish (IJ) children, and it is caused by a deficiency of the lysosomal acid lipase (LAL). Newborns with WD are healthy and active at birth but soon develop severe malnutrition symptoms and often die before 1 year. In particular, spleens, livers, bone marrows, intestines, adrenal glands, and lymph nodes accumulate harmful amounts of lipids. G87V mutation in LIPA is responsible for Wolman disorder. Some reports suggest that δ-tocopherol can reduce lipid accumulation in cholesterol storage disorders...
2023: Advances in Protein Chemistry and Structural Biology
#33
EDITORIAL
Francisca Joly, Manpreet Mundi, Rocco Barazzoni, Mette M Berger, Frederico Bozzetti, Cristina Cuerda, Palle B Jeppesen, Simon Lal, Georg Lamprecht, Kinga Szczepanek, André Van Gossum, Stéphane Schneider, Alan Shenkin, Geert Wanten, Loris Pironi
No abstract text is available yet for this article.
December 5, 2022: Clinical Nutrition
#34
REVIEW
Ryuichi Mashima, Shuji Takada
Lysosomal acid lipase (LAL) is a lysosomal enzyme essential for the degradation of cholesteryl esters through the endocytic pathway. Deficiency of the LAL enzyme encoded by the LIPA gene leads to LAL deficiency (LAL-D) (OMIM 278000), one of the lysosomal storage disorders involving 50-60 genes. Among the two disease subtypes, the severe disease subtype of LAL-D is known as Wolman disease, with typical manifestations involving hepatomegaly, splenomegaly, vomiting, diarrhea, and hematopoietic abnormalities, such as anemia...
December 8, 2022: International Journal of Molecular Sciences
#35
JOURNAL ARTICLE
Emily Haeuser, Audrey L Serfes, Michael A Cork, Mingyou Yang, Hedayat Abbastabar, E S Abhilash, Maryam Adabi, Oladimeji M Adebayo, Victor Adekanmbi, Daniel Adedayo Adeyinka, Saira Afzal, Bright Opoku Ahinkorah, Keivan Ahmadi, Muktar Beshir Ahmed, Yonas Akalu, Rufus Olusola Akinyemi, Chisom Joyqueenet Akunna, Fares Alahdab, Fahad Mashhour Alanezi, Turki M Alanzi, Kefyalew Addis Alene, Robert Kaba Alhassan, Vahid Alipour, Amir Almasi-Hashiani, Nelson Alvis-Guzman, Edward Kwabena Ameyaw, Saeed Amini, Dickson A Amugsi, Robert Ancuceanu, Davood Anvari, Seth Christopher Yaw Appiah, Jalal Arabloo, Olatunde Aremu, Mulusew A Asemahagn, Mohammad Asghari Jafarabadi, Atalel Fentahun Awedew, Beatriz Paulina Ayala Quintanilla, Martin Amogre Ayanore, Yared Asmare Aynalem, Samad Azari, Zelalem Nigussie Azene, B B Darshan, Tesleem Kayode Babalola, Atif Amin Baig, Maciej Banach, Till Winfried Bärnighausen, Arielle Wilder Bell, Akshaya Srikanth Bhagavathula, Nikha Bhardwaj, Pankaj Bhardwaj, Krittika Bhattacharyya, Ali Bijani, Zebenay Workneh Bitew, Somayeh Bohlouli, Obasanjo Afolabi Bolarinwa, Archith Boloor, Ivana Bozicevic, Zahid A Butt, Rosario Cárdenas, Felix Carvalho, Jaykaran Charan, Vijay Kumar Chattu, Mohiuddin Ahsanul Kabir Chowdhury, Dinh-Toi Chu, Richard G Cowden, Saad M A Dahlawi, Giovanni Damiani, Eugene Kofuor Maafo Darteh, Aso Mohammad Darwesh, José das Neves, Nicole Davis Weaver, Diego De Leo, Jan-Walter De Neve, Kebede Deribe, Keshab Deuba, Samath Dharmaratne, Mostafa Dianatinasab, Daniel Diaz, Alireza Didarloo, Shirin Djalalinia, Fariba Dorostkar, Eleonora Dubljanin, Bereket Duko, Maha El Tantawi, Shaimaa I El-Jaafary, Babak Eshrati, Sharareh Eskandarieh, Oghenowede Eyawo, Ifeanyi Jude Ezeonwumelu, Sayeh Ezzikouri, Farshad Farzadfar, Nazir Fattahi, Nelsensius Klau Fauk, Eduarda Fernandes, Irina Filip, Florian Fischer, Nataliya A Foigt, Masoud Foroutan, Takeshi Fukumoto, Mohamed M Gad, Abhay Motiramji Gaidhane, Birhan Gebresillassie Gebregiorgis, Ketema Bizuwork Gebremedhin, Lemma Getacher, Keyghobad Ghadiri, Ahmad Ghashghaee, Mahaveer Golechha, Mohammed Ibrahim Mohialdeen Gubari, Harish Chander Gugnani, Rafael Alves Guimarães, Mohammad Rifat Haider, Arvin Haj-Mirzaian, Samer Hamidi, Abdiwahab Hashi, Soheil Hassanipour, Hadi Hassankhani, Khezar Hayat, Claudiu Herteliu, Hung Chak Ho, Ramesh Holla, Mostafa Hosseini, Mehdi Hosseinzadeh, Bing-Fang Hwang, Segun Emmanuel Ibitoye, Olayinka Stephen Ilesanmi, Irena M Ilic, Milena D Ilic, Rakibul M Islam, Chidozie C D Iwu, Mihajlo Jakovljevic, Ravi Prakash Jha, John S Ji, Kimberly B Johnson, Nitin Joseph, Vasna Joshua, Farahnaz Joukar, Jacek Jerzy Jozwiak, Leila R Kalankesh, Rohollah Kalhor, Naser Kamyari, Tanuj Kanchan, Behzad Karami Matin, Salah Eddin Karimi, Gbenga A Kayode, Ali Kazemi Karyani, Maryam Keramati, Ejaz Ahmad Khan, Gulfaraz Khan, Md Nuruzzaman Khan, Khaled Khatab, Jagdish Khubchandani, Yun Jin Kim, Adnan Kisa, Sezer Kisa, Jacek A Kopec, Soewarta Kosen, Sindhura Lakshmi Koulmane Laxminarayana, Ai Koyanagi, Kewal Krishan, Barthelemy Kuate Defo, Nuworza Kugbey, Vaman Kulkarni, Manasi Kumar, Nithin Kumar, Dian Kusuma, Carlo La Vecchia, Dharmesh Kumar Lal, Iván Landires, Heidi Jane Larson, Savita Lasrado, Paul H Lee, Shanshan Li, Xuefeng Liu, Afshin Maleki, Preeti Malik, Mohammad Ali Mansournia, Francisco Rogerlândio Martins-Melo, Walter Mendoza, Ritesh G Menezes, Endalkachew Worku Mengesha, Tuomo J Meretoja, Tomislav Mestrovic, Andreea Mirica, Babak Moazen, Osama Mohamad, Yousef Mohammad, Abdollah Mohammadian-Hafshejani, Reza Mohammadpourhodki, Salahuddin Mohammed, Shafiu Mohammed, Ali H Mokdad, Masoud Moradi, Paula Moraga, Sumaira Mubarik, Getaneh Baye B Mulu, Lillian Mwanri, Ahamarshan Jayaraman Nagarajan, Mukhammad David Naimzada, Muhammad Naveed, Javad Nazari, Rawlance Ndejjo, Ionut Negoi, Frida N Ngalesoni, Georges Nguefack-Tsague, Josephine W Ngunjiri, Cuong Tat Nguyen, Huong Lan Thi Nguyen, Chukwudi A Nnaji, Jean Jacques Noubiap, Virginia Nuñez-Samudio, Vincent Ebuka Nwatah, Bogdan Oancea, Oluwakemi Ololade Odukoya, Andrew T Olagunju, Babayemi Oluwaseun Olakunde, Bolajoko Olubukunola Olusanya, Jacob Olusegun Olusanya, Ahmed Omar Bali, Obinna E Onwujekwe, Orish Ebere Orisakwe, Nikita Otstavnov, Stanislav S Otstavnov, Mayowa O Owolabi, P A Mahesh, Jagadish Rao Padubidri, Adrian Pana, Ashok Pandey, Seithikurippu R Pandi-Perumal, Fatemeh Pashazadeh Kan, George C Patton, Shrikant Pawar, Emmanuel K Peprah, Maarten J Postma, Liliana Preotescu, Zahiruddin Quazi Syed, Navid Rabiee, Amir Radfar, Alireza Rafiei, Fakher Rahim, Vafa Rahimi-Movaghar, Amir Masoud Rahmani, Kiana Ramezanzadeh, Juwel Rana, Chhabi Lal Ranabhat, Sowmya J Rao, David Laith Rawaf, Salman Rawaf, Reza Rawassizadeh, Lemma Demissie Regassa, Nima Rezaei, Aziz Rezapour, Mavra A Riaz, Ana Isabel Ribeiro, Jennifer M Ross, Enrico Rubagotti, Susan Fred Rumisha, Godfrey M Rwegerera, Sahar Saeedi Moghaddam, Rajesh Sagar, Biniyam Sahiledengle, Maitreyi Sahu, Marwa Rashad Salem, Hossein Samadi Kafil, Abdallah M Samy, Benn Sartorius, Brijesh Sathian, Abdul-Aziz Seidu, Amira A Shaheen, Masood Ali Shaikh, Morteza Shamsizadeh, Wondimeneh Shibabaw Shiferaw, Jae Il Shin, Roman Shrestha, Jasvinder A Singh, Valentin Yurievich Skryabin, Anna Aleksandrovna Skryabina, Shahin Soltani, Mu'awiyyah Babale Sufiyan, Takahiro Tabuchi, Eyayou Girma Tadesse, Nuno Taveira, Fisaha Haile Tesfay, Rekha Thapar, Marcos Roberto Tovani-Palone, Gebiyaw Wudie Tsegaye, Chukwuma David Umeokonkwo, Bhaskaran Unnikrishnan, Jorge Hugo Villafañe, Francesco S Violante, Bay Vo, Giang Thu Vu, Yohannes Dibaba Wado, Yasir Waheed, Richard G Wamai, Yanzhong Wang, Paul Ward, Nuwan Darshana Wickramasinghe, Katherine Wilson, Sanni Yaya, Paul Yip, Naohiro Yonemoto, Chuanhua Yu, Mikhail Sergeevich Zastrozhin, Yunquan Zhang, Zhi-Jiang Zhang, Simon I Hay, Laura Dwyer-Lindgren
BACKGROUND: Human immunodeficiency virus and acquired immune deficiency syndrome (HIV/AIDS) is still among the leading causes of disease burden and mortality in sub-Saharan Africa (SSA), and the world is not on track to meet targets set for ending the epidemic by the Joint United Nations Programme on HIV/AIDS (UNAIDS) and the United Nations Sustainable Development Goals (SDGs). Precise HIV burden information is critical for effective geographic and epidemiological targeting of prevention and treatment interventions...
December 19, 2022: BMC Medicine
#36
JOURNAL ARTICLE
Guang Yang, Siyuan Zhou, Haoqiang He, Zinuo Shen, Yongmei Liu, Jun Hu, Jie Wang
Background: According to the theory of traditional Chinese medicine, phlegm and blood stasis (PBS) is the pathological basis for coronary heart disease (CHD). This study aimed to explore the biological basis of PBS syndrome in CHD. Methods: Using a strategy that integrated RNA-seq, DIA-based proteomics, and untargeted metabolomics on 90 clinic samples, we constructed a "gene-protein-metabolite" network for CHD-PBS syndrome. We expanded the sample size and validated the differential genes and metabolites in the network through enzyme-linked immunosorbent assay...
2022: Frontiers in Pharmacology
#37
JOURNAL ARTICLE
Xinying Hong, Yicheng Chen, Marianne Barr, Michael H Gelb
BACKGROUND: Lysosomal acid lipase deficiency (LAL-D) is a phenotypic continuum between the severe Wolman disease and the attenuated cholesteryl ester storage disease (CESD). OBJECTIVE: To study if the amount of residual LAL enzymatic activity in dried blood spots (DBS) correlates with the LAL-D disease severity. METHODS: DBS from Wolman and CESD patients, LAL-D carriers, and presumably unaffected random newborns were acquired. LAL enzymatic activity in DBS were measured using a novel, highly specific LAL substrate...
December 2022: Molecular Genetics and Metabolism Reports
#38
REVIEW
Milan Kumar Lal, Rahul Kumar Tiwari, Awadhesh Kumar, Abhijit Dey, Ravinder Kumar, Dharmendra Kumar, Arvind Jaiswal, Sushil Sudhakar Changan, Pinky Raigond, Som Dutt, Satish Kumar Luthra, Sayanti Mandal, Madan Pal Singh, Vijay Paul, Brajesh Singh
Most cultivated potatoes are tetraploid, and the tuber is the main economic part that is consumed due to its calorific and nutritional values. Recent trends in climate change led to the frequent occurrence of heat and drought stress in major potato-growing regions worldwide. The optimum temperature for tuber production is 15-20 °C. High-temperature and water-deficient conditions during the growing season result in several morphological, physiological, biochemical, and molecular alterations. The morphological changes under stress conditions may affect the process of stolon formation, tuberization, and bulking, ultimately affecting the tuber yield...
October 26, 2022: Plants (Basel, Switzerland)
#39
JOURNAL ARTICLE
Rajendra Pradeepa, Lal Shreya, Ranjit Mohan Anjana, Saravanan Jebarani, Nithyanantham Kamal Raj, Srinivasan Kumar Madhan, Parthasarathy Jayaganesh, Onkar C Swami, Viswanathan Mohan
AIM: To study the frequency of iron deficiency anemia (IDA) in individuals with type 2 diabetes mellitus (T2DM) seen at tertiary diabetes care centres across India. METHODS: This is a retrospective study (January 1, 2017-December 31, 2019), which included 1137 individuals with T2DM, aged ≥18 years, for whom data on glycemic, lipid and haematological parameters were available. Anthropometric measurements were done using standardized techniques. Biochemical investigations included fasting plasma glucose[FPG], post prandial plasma glucose, HbA1c, lipids and serum ferritin and iron wherever feasible...
September 28, 2022: Diabetes & Metabolic Syndrome
#40
JOURNAL ARTICLE
Could lysosomal acid lipase enzyme activity be used for clinical follow-up in cryptogenic cirrhosis?
Engin Köse, Elçin Çağatay, Tutku Yaraş, Pelin Teke Kısa, Seminay Güler, Zümrüt Arslan Gülten, Mesut Akarsu, Yavuz Oktay, Hülya Ayar Kayalı, Nur Arslan
BACKGROUND: Cholesterol ester storage disease (CESD) is one of the rare causes that should be kept in mind in the etiology of cirrhosis. Recent studies detected that significantly reduced lysosomal acid lipase deficiency enzyme (LAL) in patients with cryptogenic cirrhosis (CC). Moreover, studies have evaluated that LAL activity is as effective as scoring systems in assessing the severity of cirrhosis. In this study, we aimed to investigate the CESD with LAL level and mutation analysis of LIPA gene in patients diagnosed with CC and to compare LAL activities between patients with CC and healthy volunteers...
August 2022: Turkish Journal of Medical Sciences
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