Publication Information

{'id': 'https://openalex.org/W2163557498', 'doi': 'https://doi.org/10.1097/01.wco.0000063762.15877.9b', 'title': 'Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis', 'display_name': 'Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis', 'publication_year': 2003, 'publication_date': '2003-04-01', 'ids': {'openalex': 'https://openalex.org/W2163557498', 'doi': 'https://doi.org/10.1097/01.wco.0000063762.15877.9b', 'mag': '2163557498', 'pmid': 'https://pubmed.ncbi.nlm.nih.gov/12644737'}, 'language': 'en', 'primary_location': {'is_oa': False, 'landing_page_url': 'https://doi.org/10.1097/01.wco.0000063762.15877.9b', 'pdf_url': None, 'source': {'id': 'https://openalex.org/S177945262', 'display_name': 'Current Opinion in Neurology', 'issn_l': '1350-7540', 'issn': ['1350-7540', '1473-6551'], 'is_oa': False, 'is_in_doaj': False, 'is_indexed_in_scopus': True, 'is_core': True, 'host_organization': 'https://openalex.org/P4310315671', 'host_organization_name': 'Lippincott Williams & Wilkins', 'host_organization_lineage': ['https://openalex.org/P4310315671', 'https://openalex.org/P4310318547'], 'host_organization_lineage_names': ['Lippincott Williams & Wilkins', 'Wolters Kluwer'], 'type': 'journal'}, 'license': None, 'license_id': None, 'version': None, 'is_accepted': False, 'is_published': False}, 'type': 'review', 'type_crossref': 'journal-article', 'indexed_in': ['crossref', 'pubmed'], 'open_access': {'is_oa': False, 'oa_status': 'closed', 'oa_url': None, 'any_repository_has_fulltext': False}, 'authorships': [{'author_position': 'first', 'author': {'id': 'https://openalex.org/A5070668450', 'display_name': 'Jonathan D. Cooper', 'orcid': 'https://orcid.org/0000-0003-1339-4750'}, 'institutions': [{'id': 'https://openalex.org/I183935753', 'display_name': "King's College London", 'ror': 'https://ror.org/0220mzb33', 'country_code': 'GB', 'type': 'education', 'lineage': ['https://openalex.org/I124357947', 'https://openalex.org/I183935753']}], 'countries': ['GB'], 'is_corresponding': True, 'raw_author_name': 'Jonathan D Cooper', 'raw_affiliation_strings': ["Pediatric Storage Disorders Laboratory, Department of Neuropathology P040, Institute of Psychiatry, King's College London, De Crespigny Park, London SE5 8AF, UK. [email protected]"], 'affiliations': [{'raw_affiliation_string': "Pediatric Storage Disorders Laboratory, Department of Neuropathology P040, Institute of Psychiatry, King's College London, De Crespigny Park, London SE5 8AF, UK. [email protected]", 'institution_ids': ['https://openalex.org/I183935753']}]}], 'institution_assertions': [], 'countries_distinct_count': 1, 'institutions_distinct_count': 1, 'corresponding_author_ids': ['https://openalex.org/A5070668450'], 'corresponding_institution_ids': ['https://openalex.org/I183935753'], 'apc_list': None, 'apc_paid': None, 'fwci': 2.306, 'has_fulltext': False, 'cited_by_count': 97, 'citation_normalized_percentile': {'value': 0.565116, 'is_in_top_1_percent': False, 'is_in_top_10_percent': False}, 'cited_by_percentile_year': {'min': 95, 'max': 96}, 'biblio': {'volume': '16', 'issue': '2', 'first_page': '121', 'last_page': '128'}, 'is_retracted': False, 'is_paratext': False, 'primary_topic': {'id': 'https://openalex.org/T10945', 'display_name': 'Lysosomal Storage Disorders Research', 'score': 1.0, 'subfield': {'id': 'https://openalex.org/subfields/2737', 'display_name': 'Physiology'}, 'field': {'id': 'https://openalex.org/fields/27', 'display_name': 'Medicine'}, 'domain': {'id': 'https://openalex.org/domains/4', 'display_name': 'Health Sciences'}}, 'topics': [{'id': 'https://openalex.org/T10945', 'display_name': 'Lysosomal Storage Disorders Research', 'score': 1.0, 'subfield': {'id': 'https://openalex.org/subfields/2737', 'display_name': 'Physiology'}, 'field': {'id': 'https://openalex.org/fields/27', 'display_name': 'Medicine'}, 'domain': {'id': 'https://openalex.org/domains/4', 'display_name': 'Health Sciences'}}, {'id': 'https://openalex.org/T13415', 'display_name': 'Calcium signaling and nucleotide metabolism', 'score': 0.9944, 'subfield': {'id': 'https://openalex.org/subfields/1314', 'display_name': 'Physiology'}, 'field': {'id': 'https://openalex.org/fields/13', 'display_name': 'Biochemistry, Genetics and Molecular Biology'}, 'domain': {'id': 'https://openalex.org/domains/1', 'display_name': 'Life Sciences'}}, {'id': 'https://openalex.org/T10617', 'display_name': 'Cellular transport and secretion', 'score': 0.9942, 'subfield': {'id': 'https://openalex.org/subfields/1307', 'display_name': 'Cell Biology'}, 'field': {'id': 'https://openalex.org/fields/13', 'display_name': 'Biochemistry, Genetics and Molecular Biology'}, 'domain': {'id': 'https://openalex.org/domains/1', 'display_name': 'Life Sciences'}}], 'keywords': [{'id': 'https://openalex.org/keywords/batten-disease', 'display_name': 'Batten disease', 'score': 0.9623748}, {'id': 'https://openalex.org/keywords/neuronal-ceroid-lipofuscinosis', 'display_name': 'Neuronal ceroid lipofuscinosis', 'score': 0.9051872}], 'concepts': [{'id': 'https://openalex.org/C2778230176', 'wikidata': 'https://www.wikidata.org/wiki/Q1753778', 'display_name': 'Batten disease', 'level': 3, 'score': 0.9623748}, {'id': 'https://openalex.org/C2778650512', 'wikidata': 'https://www.wikidata.org/wiki/Q4358039', 'display_name': 'Neuronal ceroid lipofuscinosis', 'level': 3, 'score': 0.9051872}, {'id': 'https://openalex.org/C169760540', 'wikidata': 'https://www.wikidata.org/wiki/Q207011', 'display_name': 'Neuroscience', 'level': 1, 'score': 0.71000093}, {'id': 'https://openalex.org/C86803240', 'wikidata': 'https://www.wikidata.org/wiki/Q420', 'display_name': 'Biology', 'level': 0, 'score': 0.61816585}, {'id': 'https://openalex.org/C2779134260', 'wikidata': 'https://www.wikidata.org/wiki/Q12136', 'display_name': 'Disease', 'level': 2, 'score': 0.5136013}, {'id': 'https://openalex.org/C104317684', 'wikidata': 'https://www.wikidata.org/wiki/Q7187', 'display_name': 'Gene', 'level': 2, 'score': 0.35740566}, {'id': 'https://openalex.org/C54355233', 'wikidata': 'https://www.wikidata.org/wiki/Q7162', 'display_name': 'Genetics', 'level': 1, 'score': 0.26356962}, {'id': 'https://openalex.org/C71924100', 'wikidata': 'https://www.wikidata.org/wiki/Q11190', 'display_name': 'Medicine', 'level': 0, 'score': 0.17691725}, {'id': 'https://openalex.org/C142724271', 'wikidata': 'https://www.wikidata.org/wiki/Q7208', 'display_name': 'Pathology', 'level': 1, 'score': 0.14782381}], 'mesh': [{'descriptor_ui': 'D009154', 'descriptor_name': 'Mutation', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': True}, {'descriptor_ui': 'D009472', 'descriptor_name': 'Neuronal Ceroid-Lipofuscinoses', 'qualifier_ui': 'Q000235', 'qualifier_name': 'genetics', 'is_major_topic': True}, {'descriptor_ui': 'D000818', 'descriptor_name': 'Animals', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D016923', 'descriptor_name': 'Cell Death', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D004195', 'descriptor_name': 'Disease Models, Animal', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D006801', 'descriptor_name': 'Humans', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D016464', 'descriptor_name': 'Lysosomal Storage Diseases', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D016464', 'descriptor_name': 'Lysosomal Storage Diseases', 'qualifier_ui': 'Q000503', 'qualifier_name': 'physiopathology', 'is_major_topic': False}, {'descriptor_ui': 'D009457', 'descriptor_name': 'Neuroglia', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D009457', 'descriptor_name': 'Neuroglia', 'qualifier_ui': 'Q000378', 'qualifier_name': 'metabolism', 'is_major_topic': False}, {'descriptor_ui': 'D015213', 'descriptor_name': 'Neuroimmunomodulation', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D009472', 'descriptor_name': 'Neuronal Ceroid-Lipofuscinoses', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D009472', 'descriptor_name': 'Neuronal Ceroid-Lipofuscinoses', 'qualifier_ui': 'Q000378', 'qualifier_name': 'metabolism', 'is_major_topic': False}, {'descriptor_ui': 'D009472', 'descriptor_name': 'Neuronal Ceroid-Lipofuscinoses', 'qualifier_ui': 'Q000473', 'qualifier_name': 'pathology', 'is_major_topic': False}, {'descriptor_ui': 'D009472', 'descriptor_name': 'Neuronal Ceroid-Lipofuscinoses', 'qualifier_ui': 'Q000503', 'qualifier_name': 'physiopathology', 'is_major_topic': False}, {'descriptor_ui': 'D009472', 'descriptor_name': 'Neuronal Ceroid-Lipofuscinoses', 'qualifier_ui': 'Q000628', 'qualifier_name': 'therapy', 'is_major_topic': False}, {'descriptor_ui': 'D021381', 'descriptor_name': 'Protein Transport', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}], 'locations_count': 2, 'locations': [{'is_oa': False, 'landing_page_url': 'https://doi.org/10.1097/01.wco.0000063762.15877.9b', 'pdf_url': None, 'source': {'id': 'https://openalex.org/S177945262', 'display_name': 'Current Opinion in Neurology', 'issn_l': '1350-7540', 'issn': ['1350-7540', '1473-6551'], 'is_oa': False, 'is_in_doaj': False, 'is_indexed_in_scopus': True, 'is_core': True, 'host_organization': 'https://openalex.org/P4310315671', 'host_organization_name': 'Lippincott Williams & Wilkins', 'host_organization_lineage': ['https://openalex.org/P4310315671', 'https://openalex.org/P4310318547'], 'host_organization_lineage_names': ['Lippincott Williams & Wilkins', 'Wolters Kluwer'], 'type': 'journal'}, 'license': None, 'license_id': None, 'version': None, 'is_accepted': False, 'is_published': False}, {'is_oa': False, 'landing_page_url': 'https://pubmed.ncbi.nlm.nih.gov/12644737', 'pdf_url': None, 'source': {'id': 'https://openalex.org/S4306525036', 'display_name': 'PubMed', 'issn_l': None, 'issn': None, 'is_oa': False, 'is_in_doaj': False, 'is_indexed_in_scopus': False, 'is_core': False, 'host_organization': 'https://openalex.org/I1299303238', 'host_organization_name': 'National Institutes of Health', 'host_organization_lineage': ['https://openalex.org/I1299303238'], 'host_organization_lineage_names': ['National Institutes of Health'], 'type': 'repository'}, 'license': None, 'license_id': None, 'version': None, 'is_accepted': False, 'is_published': False}], 'best_oa_location': None, 'sustainable_development_goals': [], 'grants': [], 'datasets': [], 'versions': [], 'referenced_works_count': 74, 'referenced_works': ['https://openalex.org/W117537364', 'https://openalex.org/W1597721823', 'https://openalex.org/W1651773168', 'https://openalex.org/W1797956629', 'https://openalex.org/W1972594822', 'https://openalex.org/W1976334979', 'https://openalex.org/W1988967065', 'https://openalex.org/W1989262042', 'https://openalex.org/W1990911694', 'https://openalex.org/W1991439335', 'https://openalex.org/W1995562819', 'https://openalex.org/W2000838235', 'https://openalex.org/W2003777400', 'https://openalex.org/W2004999768', 'https://openalex.org/W2006021227', 'https://openalex.org/W2008524970', 'https://openalex.org/W2011933633', 'https://openalex.org/W2012796647', 'https://openalex.org/W2019404480', 'https://openalex.org/W2019965714', 'https://openalex.org/W2021311269', 'https://openalex.org/W2026657765', 'https://openalex.org/W2034846875', 'https://openalex.org/W2035426809', 'https://openalex.org/W2038852344', 'https://openalex.org/W2040516771', 'https://openalex.org/W2041358572', 'https://openalex.org/W2045480080', 'https://openalex.org/W2047079246', 'https://openalex.org/W2056882920', 'https://openalex.org/W2058677460', 'https://openalex.org/W2058793126', 'https://openalex.org/W2059092093', 'https://openalex.org/W2059277097', 'https://openalex.org/W2060341674', 'https://openalex.org/W2060427658', 'https://openalex.org/W2060462371', 'https://openalex.org/W2071243924', 'https://openalex.org/W2074508409', 'https://openalex.org/W2076584350', 'https://openalex.org/W2078982705', 'https://openalex.org/W2079469770', 'https://openalex.org/W2081673312', 'https://openalex.org/W2082925865', 'https://openalex.org/W2084548862', 'https://openalex.org/W2088514390', 'https://openalex.org/W2089592662', 'https://openalex.org/W2093431846', 'https://openalex.org/W2094954724', 'https://openalex.org/W2097005698', 'https://openalex.org/W2098012937', 'https://openalex.org/W2102335037', 'https://openalex.org/W2108155784', 'https://openalex.org/W2125030425', 'https://openalex.org/W2126213759', 'https://openalex.org/W2132540143', 'https://openalex.org/W2144968601', 'https://openalex.org/W2145231670', 'https://openalex.org/W2146368604', 'https://openalex.org/W2146715646', 'https://openalex.org/W2152773842', 'https://openalex.org/W2156501544', 'https://openalex.org/W2156561885', 'https://openalex.org/W2156881505', 'https://openalex.org/W2163711162', 'https://openalex.org/W2164672515', 'https://openalex.org/W2166500502', 'https://openalex.org/W2168540074', 'https://openalex.org/W2218419942', 'https://openalex.org/W2326834377', 'https://openalex.org/W2340067247', 'https://openalex.org/W2412556252', 'https://openalex.org/W2417637566', 'https://openalex.org/W257126876'], 'related_works': ['https://openalex.org/W82223775', 'https://openalex.org/W3081783566', 'https://openalex.org/W3016158054', 'https://openalex.org/W2317527551', 'https://openalex.org/W2166453547', 'https://openalex.org/W2163557498', 'https://openalex.org/W2126741328', 'https://openalex.org/W2124523908', 'https://openalex.org/W2082887407', 'https://openalex.org/W1974168805'], 'abstract_inverted_index': {'Purpose': [0], 'of': [1, 5, 52, 56, 63, 78, 82, 100, 114, 150, 160, 170, 174, 199, 202, 213, 228], 'review': [2, 36], 'The': [3, 158], 'identification': [4], 'genes': [6, 122], 'mutated': [7], 'in': [8, 39, 47, 123, 241], 'the': [9, 17, 50, 61, 79, 97, 120, 124, 145, 154, 171, 200, 207, 210, 225, 229], 'neuronal': [10, 57, 83, 203], 'ceroid': [11, 58, 84, 204], 'lipofuscinoses': [12], 'has': [13, 166], 'accelerated': [14], 'research': [15], 'into': [16, 69, 191], 'mechanisms': [18, 193], 'that': [19, 236], 'underlie': [20], 'these': [21, 132, 151], 'fatal': [22], 'autosomal': [23], 'recessive': [24], 'storage': [25], 'disorders,': [26], 'which': [27], 'are': [28, 93, 194], 'often': [29], 'referred': [30], 'to': [31, 66, 95, 117, 223, 232], 'as': [32], 'Batten': [33], 'disease.': [34], 'This': [35], 'summarizes': [37], 'progress': [38], 'this': [40, 106], 'field': [41], 'since': [42], 'October': [43], '2001,': [44], 'describing': [45], 'advances': [46], 'cell': [48], 'biology,': [49], 'characterization': [51, 177], 'new': [53], 'animal': [54, 164], 'models': [55, 165], 'lipofuscinosis,': [59], 'and': [60, 90, 104, 127, 147, 162, 180, 186, 231], 'impact': [62, 201], 'novel': [64], 'methodology': [65], 'reveal': [67], 'insights': [68], 'its': [70], 'pathogenesis.': [71], 'Recent': [72], 'findings': [73], 'Gene': [74], 'products': [75], 'for': [76, 119, 131, 137], 'six': [77], 'eight': [80], 'forms': [81], 'lipofuscinosis': [85, 205], 'have': [86], 'now': [87], 'been': [88], 'discovered,': [89], 'concerted': [91], 'efforts': [92], 'underway': [94], 'understand': [96], 'normal': [98], 'biology': [99], 'each': [101], 'gene': [102], 'product': [103], 'how': [105], 'may': [107, 143], 'be': [108, 220, 239], 'altered': [109], 'by': [110, 178, 184], 'mutation.': [111], 'Several': [112], 'lines': [113], 'evidence': [115], 'point': [116], 'functions': [118], 'CLN': [121], 'endosomal-lysosomal': [125], 'system,': [126], 'suggest': [128], 'neuron-specific': [129], 'roles': [130], 'proteins.': [133], 'Indeed,': [134], 'a': [135, 196], 'requirement': [136], 'appropriate': [138], 'protein': [139], 'trafficking': [140], 'within': [141], 'neurons': [142], 'explain': [144], 'profound': [146], 'selective': [148], 'effects': [149, 173], 'disorders': [152], 'upon': [153, 206], 'central': [155], 'nervous': [156], 'system.': [157], 'development': [159], 'mouse': [161], 'large': [163], 'enabled': [167], 'comparative': [168], 'studies': [169], 'progressive': [172], 'disease,': [175], 'including': [176], 'morphological': [179], 'biochemical': [181], 'means': [182], 'supplemented': [183], 'metabonomic': [185], 'microarray': [187], 'techniques.': [188], 'Summary': [189], 'Insights': [190], 'disease': [192], 'building': [195], 'detailed': [197], 'profile': [198], 'brain.': [208], 'With': [209], 'eventual': [211], 'aim': [212], 'developing': [214], 'successful': [215], 'therapeutic': [216], 'strategies,': [217], 'it': [218], 'will': [219], 'equally': [221], 'important': [222], 'characterize': [224], 'clinical': [226, 242], 'progression': [227], 'disorder,': [230], 'identify': [233], 'quantifiable': [234], 'endpoints': [235], 'can': [237], 'ultimately': [238], 'used': [240], 'trials.': [243]}, 'abstract_inverted_index_v3': None, 'cited_by_api_url': 'https://api.openalex.org/works?filter=cites:W2163557498', 'counts_by_year': [{'year': 2022, 'cited_by_count': 1}, {'year': 2020, 'cited_by_count': 2}, {'year': 2019, 'cited_by_count': 2}, {'year': 2018, 'cited_by_count': 1}, {'year': 2017, 'cited_by_count': 3}, {'year': 2016, 'cited_by_count': 2}, {'year': 2015, 'cited_by_count': 2}, {'year': 2013, 'cited_by_count': 10}, {'year': 2012, 'cited_by_count': 1}], 'updated_date': '2025-01-22T04:27:09.783570', 'created_date': '2016-06-24'}