{"id":111586,"date":"2025-12-25T14:47:53","date_gmt":"2025-12-25T14:47:53","guid":{"rendered":"https:\/\/advbiomart.sytech.site\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/"},"modified":"2025-12-25T14:47:54","modified_gmt":"2025-12-25T14:47:54","slug":"recombinant-human-alpha-galactosidase-a-gla-acp04293","status":"publish","type":"product","link":"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/","title":{"rendered":"Recombinant Human Alpha-galactosidase A (GLA)"},"content":{"rendered":"","protected":false},"excerpt":{"rendered":"

Alpha-galactosidase A (GLA) is a crucial enzyme involved in lysosomal function and lipid metabolism. GLA is responsible for hydrolyzing the terminal alpha-galactosyl moiety from glycoconjugates [1]. Mutations in the GLA gene lead to Fabry disease, a rare X-linked disorder characterized by deficient activity of GLA, resulting in the intracellular accumulation of enzyme substrates inside lysosomes [2]. This accumulation, particularly of globotriaosylceramide-3 (Gb3), is specific to Fabry disease with classical mutations and is associated with small fiber neuropathy [3].
\nFabry disease can present with symptoms mimicking hypertrophic cardiomyopathy, emphasizing the importance of genetic screening to establish a diagnosis, especially in women [4]. The absence or deficiency of GLA enzyme due to mutations results in multiorgan glycosphingolipid accumulations, leading to various clinical manifestations [5]. Chaperone therapy has been explored in Fabry disease to address the underlying enzyme deficiency [6].
\nThe cardiovascular phenotype in Fabry disease has been linked to residual GLA activity, which may influence disease progression and the manifestation of clinical signs [7]. Dysregulation of immune response mediators and pain-related ion channels has been associated with pain-like behavior in Fabry disease, highlighting the complex interplay between GLA mutations and symptomatology [8]. Additionally, cornea verticillata has been reported in classical Fabry disease cases, further underlining the diverse systemic manifestations of GLA mutations.
\nReferences:[1] S. Wu, L. Xiang, J. Geng, M. Zhang, N. Xie, & X. Su, “Hydroxychloroquine-induced renal phospholipidosis resembling fabry disease in undifferentiated connective tissue disease: a case report”, World Journal of Clinical Cases, vol. 7, no. 24, p. 4377-4383, 2019. https:\/\/doi.org\/10.12998\/wjcc.v7.i24.4377[2] R. Liguori, A. Incensi, S. Pasqua, R. Mignani, E. Fileccia, M. Santostefanoet al., “Skin globotriaosylceramide 3 deposits are specific to fabry disease with classical mutations and associated with small fibre neuropathy”, Plos One, vol. 12, no. 7, p. e0180581, 2017. https:\/\/doi.org\/10.1371\/journal.pone.0180581[3] O. Havndrup, M. Christiansen, B. Stoevring, M. Jensen, J. Hoffman-Bang, P. Andersenet al., “Fabry disease mimicking hypertrophic cardiomyopathy: genetic screening needed for establishing the diagnosis in women”, European Journal of Heart Failure, vol. 12, no. 6, p. 535-540, 2010. https:\/\/doi.org\/10.1093\/eurjhf\/hfq073[4] S. Gaballa, A. AlJaf, J. Lindsay, K. Patel, & K. Hlaing, “Rare etiology of renal failure in a 25-year-old caucasian man: fabry disease with a novel mutation of gla gene”, Cureus, 2020. https:\/\/doi.org\/10.7759\/cureus.9136[5] F. Weidemann, A. Jovanovi?, K. Herrmann, & ?. Vardarli, “Chaperone therapy in fabry disease”, International Journal of Molecular Sciences, vol. 23, no. 3, p. 1887, 2022. https:\/\/doi.org\/10.3390\/ijms23031887[6] D. Sorriento and G. Iaccarino, “The cardiovascular phenotype in fabry disease: new findings in the research field”, International Journal of Molecular Sciences, vol. 22, no. 3, p. 1331, 2021. https:\/\/doi.org\/10.3390\/ijms22031331[7] M. Spitzel, E. Wagner, M. Breyer, D. Henniger, M. Bayin, L. Hofmannet al., “Dysregulation of immune response mediators and pain-related ion channels is associated with pain-like behavior in the gla ko mouse model of fabry disease”, Cells, vol. 11, no. 11, p. 1730, 2022. https:\/\/doi.org\/10.3390\/cells11111730[8] H. Hewavitharana, E. Jasinge, H. Abeysekera, & J. Wanigasinghe, “Cornea verticillata in classical fabry disease, first from sri lanka: a case report”, BMC Pediatrics, vol. 20, no. 1, 2020. https:\/\/doi.org\/10.1186\/s12887-020-02237-z<\/p>\n","protected":false},"featured_media":0,"comment_status":"open","ping_status":"closed","template":"","meta":{"_acf_changed":false},"product_brand":[],"product_cat":[168834,18],"product_tag":[70602],"class_list":["post-111586","product","type-product","status-publish","product_cat-proteins","product_cat-recombinant-proteins","product_tag-gla","first","instock","shipping-taxable","product-type-simple"],"acf":[],"yoast_head":"\nRecombinant Human Alpha-galactosidase A (GLA) - AbTrivia<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/\" \/>\n<meta property=\"og:locale\" content=\"de_DE\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Recombinant Human Alpha-galactosidase A (GLA) - AbTrivia\" \/>\n<meta property=\"og:description\" content=\"Alpha-galactosidase A (GLA) is a crucial enzyme involved in lysosomal function and lipid metabolism. GLA is responsible for hydrolyzing the terminal alpha-galactosyl moiety from glycoconjugates [1]. Mutations in the GLA gene lead to Fabry disease, a rare X-linked disorder characterized by deficient activity of GLA, resulting in the intracellular accumulation of enzyme substrates inside lysosomes [2]. This accumulation, particularly of globotriaosylceramide-3 (Gb3), is specific to Fabry disease with classical mutations and is associated with small fiber neuropathy [3]. Fabry disease can present with symptoms mimicking hypertrophic cardiomyopathy, emphasizing the importance of genetic screening to establish a diagnosis, especially in women [4]. The absence or deficiency of GLA enzyme due to mutations results in multiorgan glycosphingolipid accumulations, leading to various clinical manifestations [5]. Chaperone therapy has been explored in Fabry disease to address the underlying enzyme deficiency [6]. The cardiovascular phenotype in Fabry disease has been linked to residual GLA activity, which may influence disease progression and the manifestation of clinical signs [7]. Dysregulation of immune response mediators and pain-related ion channels has been associated with pain-like behavior in Fabry disease, highlighting the complex interplay between GLA mutations and symptomatology [8]. Additionally, cornea verticillata has been reported in classical Fabry disease cases, further underlining the diverse systemic manifestations of GLA mutations. References:[1] S. Wu, L. Xiang, J. Geng, M. Zhang, N. Xie, & X. Su, "Hydroxychloroquine-induced renal phospholipidosis resembling fabry disease in undifferentiated connective tissue disease: a case report", World Journal of Clinical Cases, vol. 7, no. 24, p. 4377-4383, 2019. https:\/\/doi.org\/10.12998\/wjcc.v7.i24.4377[2] R. Liguori, A. Incensi, S. Pasqua, R. Mignani, E. Fileccia, M. Santostefanoet al., "Skin globotriaosylceramide 3 deposits are specific to fabry disease with classical mutations and associated with small fibre neuropathy", Plos One, vol. 12, no. 7, p. e0180581, 2017. https:\/\/doi.org\/10.1371\/journal.pone.0180581[3] O. Havndrup, M. Christiansen, B. Stoevring, M. Jensen, J. Hoffman-Bang, P. Andersenet al., "Fabry disease mimicking hypertrophic cardiomyopathy: genetic screening needed for establishing the diagnosis in women", European Journal of Heart Failure, vol. 12, no. 6, p. 535-540, 2010. https:\/\/doi.org\/10.1093\/eurjhf\/hfq073[4] S. Gaballa, A. AlJaf, J. Lindsay, K. Patel, & K. Hlaing, "Rare etiology of renal failure in a 25-year-old caucasian man: fabry disease with a novel mutation of gla gene", Cureus, 2020. https:\/\/doi.org\/10.7759\/cureus.9136[5] F. Weidemann, A. Jovanovi?, K. Herrmann, & ?. Vardarli, "Chaperone therapy in fabry disease", International Journal of Molecular Sciences, vol. 23, no. 3, p. 1887, 2022. https:\/\/doi.org\/10.3390\/ijms23031887[6] D. Sorriento and G. Iaccarino, "The cardiovascular phenotype in fabry disease: new findings in the research field", International Journal of Molecular Sciences, vol. 22, no. 3, p. 1331, 2021. https:\/\/doi.org\/10.3390\/ijms22031331[7] M. Spitzel, E. Wagner, M. Breyer, D. Henniger, M. Bayin, L. Hofmannet al., "Dysregulation of immune response mediators and pain-related ion channels is associated with pain-like behavior in the gla ko mouse model of fabry disease", Cells, vol. 11, no. 11, p. 1730, 2022. https:\/\/doi.org\/10.3390\/cells11111730[8] H. Hewavitharana, E. Jasinge, H. Abeysekera, & J. Wanigasinghe, "Cornea verticillata in classical fabry disease, first from sri lanka: a case report", BMC Pediatrics, vol. 20, no. 1, 2020. https:\/\/doi.org\/10.1186\/s12887-020-02237-z\" \/>\n<meta property=\"og:url\" content=\"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/\" \/>\n<meta property=\"og:site_name\" content=\"AbTrivia\" \/>\n<meta property=\"article:modified_time\" content=\"2025-12-25T14:47:54+00:00\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/\",\"url\":\"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/\",\"name\":\"Recombinant Human Alpha-galactosidase A (GLA) - AbTrivia\",\"isPartOf\":{\"@id\":\"https:\/\/abtriva.com\/cn\/#website\"},\"datePublished\":\"2025-12-25T14:47:53+00:00\",\"dateModified\":\"2025-12-25T14:47:54+00:00\",\"breadcrumb\":{\"@id\":\"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/#breadcrumb\"},\"inLanguage\":\"de_DE\",\"potentialAction\":[{\"@type\":\"ReadAction\",\"target\":[\"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/\"]}]},{\"@type\":\"BreadcrumbList\",\"@id\":\"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/#breadcrumb\",\"itemListElement\":[{\"@type\":\"ListItem\",\"position\":1,\"name\":\"Home\",\"item\":\"https:\/\/www.abtriva.com\/de\/\"},{\"@type\":\"ListItem\",\"position\":2,\"name\":\"Products\",\"item\":\"https:\/\/www.abtriva.com\/de\/shop\/\"},{\"@type\":\"ListItem\",\"position\":3,\"name\":\"Proteins\",\"item\":\"https:\/\/www.abtriva.com\/de\/category\/proteins\/\"},{\"@type\":\"ListItem\",\"position\":4,\"name\":\"Recombinant Proteins\",\"item\":\"https:\/\/www.abtriva.com\/de\/category\/proteins\/recombinant-proteins\/\"},{\"@type\":\"ListItem\",\"position\":5,\"name\":\"Recombinant Human Alpha-galactosidase A (GLA)\"}]},{\"@type\":\"WebSite\",\"@id\":\"https:\/\/abtriva.com\/cn\/#website\",\"url\":\"https:\/\/abtriva.com\/cn\/\",\"name\":\"AbTrivia\",\"description\":\"\",\"potentialAction\":[{\"@type\":\"SearchAction\",\"target\":{\"@type\":\"EntryPoint\",\"urlTemplate\":\"https:\/\/abtriva.com\/cn\/?s={search_term_string}\"},\"query-input\":{\"@type\":\"PropertyValueSpecification\",\"valueRequired\":true,\"valueName\":\"search_term_string\"}}],\"inLanguage\":\"de_DE\"}]}<\/script>\n<!-- \/ Yoast SEO plugin. -->","yoast_head_json":{"title":"Recombinant Human Alpha-galactosidase A (GLA) - AbTrivia","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/","og_locale":"de_DE","og_type":"article","og_title":"Recombinant Human Alpha-galactosidase A (GLA) - AbTrivia","og_description":"Alpha-galactosidase A (GLA) is a crucial enzyme involved in lysosomal function and lipid metabolism. GLA is responsible for hydrolyzing the terminal alpha-galactosyl moiety from glycoconjugates [1]. Mutations in the GLA gene lead to Fabry disease, a rare X-linked disorder characterized by deficient activity of GLA, resulting in the intracellular accumulation of enzyme substrates inside lysosomes [2]. This accumulation, particularly of globotriaosylceramide-3 (Gb3), is specific to Fabry disease with classical mutations and is associated with small fiber neuropathy [3]. Fabry disease can present with symptoms mimicking hypertrophic cardiomyopathy, emphasizing the importance of genetic screening to establish a diagnosis, especially in women [4]. The absence or deficiency of GLA enzyme due to mutations results in multiorgan glycosphingolipid accumulations, leading to various clinical manifestations [5]. Chaperone therapy has been explored in Fabry disease to address the underlying enzyme deficiency [6]. The cardiovascular phenotype in Fabry disease has been linked to residual GLA activity, which may influence disease progression and the manifestation of clinical signs [7]. Dysregulation of immune response mediators and pain-related ion channels has been associated with pain-like behavior in Fabry disease, highlighting the complex interplay between GLA mutations and symptomatology [8]. Additionally, cornea verticillata has been reported in classical Fabry disease cases, further underlining the diverse systemic manifestations of GLA mutations. References:[1] S. Wu, L. Xiang, J. Geng, M. Zhang, N. Xie, & X. Su, \"Hydroxychloroquine-induced renal phospholipidosis resembling fabry disease in undifferentiated connective tissue disease: a case report\", World Journal of Clinical Cases, vol. 7, no. 24, p. 4377-4383, 2019. https:\/\/doi.org\/10.12998\/wjcc.v7.i24.4377[2] R. Liguori, A. Incensi, S. Pasqua, R. Mignani, E. Fileccia, M. Santostefanoet al., \"Skin globotriaosylceramide 3 deposits are specific to fabry disease with classical mutations and associated with small fibre neuropathy\", Plos One, vol. 12, no. 7, p. e0180581, 2017. https:\/\/doi.org\/10.1371\/journal.pone.0180581[3] O. Havndrup, M. Christiansen, B. Stoevring, M. Jensen, J. Hoffman-Bang, P. Andersenet al., \"Fabry disease mimicking hypertrophic cardiomyopathy: genetic screening needed for establishing the diagnosis in women\", European Journal of Heart Failure, vol. 12, no. 6, p. 535-540, 2010. https:\/\/doi.org\/10.1093\/eurjhf\/hfq073[4] S. Gaballa, A. AlJaf, J. Lindsay, K. Patel, & K. Hlaing, \"Rare etiology of renal failure in a 25-year-old caucasian man: fabry disease with a novel mutation of gla gene\", Cureus, 2020. https:\/\/doi.org\/10.7759\/cureus.9136[5] F. Weidemann, A. Jovanovi?, K. Herrmann, & ?. Vardarli, \"Chaperone therapy in fabry disease\", International Journal of Molecular Sciences, vol. 23, no. 3, p. 1887, 2022. https:\/\/doi.org\/10.3390\/ijms23031887[6] D. Sorriento and G. Iaccarino, \"The cardiovascular phenotype in fabry disease: new findings in the research field\", International Journal of Molecular Sciences, vol. 22, no. 3, p. 1331, 2021. https:\/\/doi.org\/10.3390\/ijms22031331[7] M. Spitzel, E. Wagner, M. Breyer, D. Henniger, M. Bayin, L. Hofmannet al., \"Dysregulation of immune response mediators and pain-related ion channels is associated with pain-like behavior in the gla ko mouse model of fabry disease\", Cells, vol. 11, no. 11, p. 1730, 2022. https:\/\/doi.org\/10.3390\/cells11111730[8] H. Hewavitharana, E. Jasinge, H. Abeysekera, & J. Wanigasinghe, \"Cornea verticillata in classical fabry disease, first from sri lanka: a case report\", BMC Pediatrics, vol. 20, no. 1, 2020. https:\/\/doi.org\/10.1186\/s12887-020-02237-z","og_url":"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/","og_site_name":"AbTrivia","article_modified_time":"2025-12-25T14:47:54+00:00","twitter_card":"summary_large_image","schema":{"@context":"https:\/\/schema.org","@graph":[{"@type":"WebPage","@id":"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/","url":"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/","name":"Recombinant Human Alpha-galactosidase A (GLA) - AbTrivia","isPartOf":{"@id":"https:\/\/abtriva.com\/cn\/#website"},"datePublished":"2025-12-25T14:47:53+00:00","dateModified":"2025-12-25T14:47:54+00:00","breadcrumb":{"@id":"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/#breadcrumb"},"inLanguage":"de_DE","potentialAction":[{"@type":"ReadAction","target":["https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/"]}]},{"@type":"BreadcrumbList","@id":"https:\/\/www.abtriva.com\/de\/product\/recombinant-human-alpha-galactosidase-a-gla-acp04293\/#breadcrumb","itemListElement":[{"@type":"ListItem","position":1,"name":"Home","item":"https:\/\/www.abtriva.com\/de\/"},{"@type":"ListItem","position":2,"name":"Products","item":"https:\/\/www.abtriva.com\/de\/shop\/"},{"@type":"ListItem","position":3,"name":"Proteins","item":"https:\/\/www.abtriva.com\/de\/category\/proteins\/"},{"@type":"ListItem","position":4,"name":"Recombinant Proteins","item":"https:\/\/www.abtriva.com\/de\/category\/proteins\/recombinant-proteins\/"},{"@type":"ListItem","position":5,"name":"Recombinant Human Alpha-galactosidase A (GLA)"}]},{"@type":"WebSite","@id":"https:\/\/abtriva.com\/cn\/#website","url":"https:\/\/abtriva.com\/cn\/","name":"AbTrivia","description":"","potentialAction":[{"@type":"SearchAction","target":{"@type":"EntryPoint","urlTemplate":"https:\/\/abtriva.com\/cn\/?s={search_term_string}"},"query-input":{"@type":"PropertyValueSpecification","valueRequired":true,"valueName":"search_term_string"}}],"inLanguage":"de_DE"}]}},"_links":{"self":[{"href":"https:\/\/www.abtriva.com\/de\/wp-json\/wp\/v2\/product\/111586","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.abtriva.com\/de\/wp-json\/wp\/v2\/product"}],"about":[{"href":"https:\/\/www.abtriva.com\/de\/wp-json\/wp\/v2\/types\/product"}],"replies":[{"embeddable":true,"href":"https:\/\/www.abtriva.com\/de\/wp-json\/wp\/v2\/comments?post=111586"}],"wp:attachment":[{"href":"https:\/\/www.abtriva.com\/de\/wp-json\/wp\/v2\/media?parent=111586"}],"wp:term":[{"taxonomy":"product_brand","embeddable":true,"href":"https:\/\/www.abtriva.com\/de\/wp-json\/wp\/v2\/product_brand?post=111586"},{"taxonomy":"product_cat","embeddable":true,"href":"https:\/\/www.abtriva.com\/de\/wp-json\/wp\/v2\/product_cat?post=111586"},{"taxonomy":"product_tag","embeddable":true,"href":"https:\/\/www.abtriva.com\/de\/wp-json\/wp\/v2\/product_tag?post=111586"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}