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Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
Catalog Number: BOSSBS-13147R-A750
UOM: 1 * 100 µl
Supplier: Bioss


Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
Catalog Number: BOSSBS-13147R-HRP
UOM: 1 * 100 µl
Supplier: Bioss


Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
Catalog Number: BOSSBS-9631R-CY5
UOM: 1 * 100 µl
Supplier: Bioss


Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
Catalog Number: BOSSBS-9631R-CY7
UOM: 1 * 100 µl
Supplier: Bioss


Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
Catalog Number: BOSSBS-9631R-A488
UOM: 1 * 100 µl
Supplier: Bioss


Description: Involvement in disease; Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterised by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
Catalog Number: BOSSBS-9631R-A750
UOM: 1 * 100 µl
Supplier: Bioss


Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
Catalog Number: BOSSBS-13147R-A647
UOM: 1 * 100 µl
Supplier: Bioss


Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
Catalog Number: BOSSBS-13147R-A680
UOM: 1 * 100 µl
Supplier: Bioss


Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
Catalog Number: BOSSBS-9631R-A350
UOM: 1 * 100 µl
Supplier: Bioss


Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
Catalog Number: BOSSBS-9631R-HRP
UOM: 1 * 100 µl
Supplier: Bioss


Description: Beta-defensins (also designated BD, and hBD in human) are small cationic peptides with broad-spectrum antimicrobial activity. Produced in mucosal epithelia and neutrophils of several species, Beta-defensins are developmentally regulated. Human b-defensin 2 is locally regulated by inflammation and is the first member of the b-defensin family that is locally inducible by inflammation. The murine homolog of human b-defensin 2, which is called b-defensin 3, is present in the respiratory system and in low levels in the epithelial cells of the intestine and lung. The unique murine b-defensin 2 (Defb2) is not expressed in airways of untreated mice, but is upregulated in the airways by lipopolysaccharide and may contribute to host defense at the mucosal surface of the airways
Catalog Number: BOSSBS-4307R-A555
UOM: 1 * 100 µl
Supplier: Bioss


Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
Catalog Number: BOSSBS-9631R-FITC
UOM: 1 * 100 µl
Supplier: Bioss


Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
Catalog Number: BOSSBS-9631R-CY5.5
UOM: 1 * 100 µl
Supplier: Bioss


Description: FNBP1 is a 617 amino acid protein that localizes to a variety of locations within the cell, including the cytoplasm, cytoskeleton, lysosome and the cell cortex, and contains one FCH domain, one REM repeat and one SH3 domain. Expressed at high levels in respiratory, reproductive and urinary systems, as well as in brown adipose tissue and epithelial cells of the gastrointestinal tract, FNBP1 interacts with Rho 7 and links the Actin cytoskeleton with Rho 7 signaling, playing a crucial role in membrane tubulation and cytoskeletal reorganization during endocytosis. Additionally, FNBP1, which exists as four alternatively spliced isoforms, enhances Actin polymerization and promotes membrane invagination and the formation of tubules. Chromosomal aberrations in the FNBP1 gene are associated with acute leukemias, suggesting a role for defective FNBP1 in carcinogenesis.
Catalog Number: BOSSBS-13147R-A555
UOM: 1 * 100 µl
Supplier: Bioss


Description: OxiTop®-IDS (Intelligent Digital Sensors) is a modern measuring instrument for use in all kinds of respirometric studies. The versatile OxiTop®-IDS is suitable for both aerobic or anaerobic examinations. All heads can be used independently from any meter for normal BOD measurements between one and seven days.
Catalog Number: 705-1185
UOM: 1 * 1 items
Supplier: WTW


Description: Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
Catalog Number: BOSSBS-9631R-CY3
UOM: 1 * 100 µl
Supplier: Bioss


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