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Katalógové číslo: (BOSSBS-3616R-A680)
Dodávateľ: BIOSS INC
Opis: NCF4 is a cytosolic oxidase associated protein. It is a component of the nicotinamide adenine dinucleotide phosphate oxidase, which mediates down regulation of NADPH oxidase through interactions with its SH3 domain. NCF4 is associated with p67 phox but is absent in patients with chronic granulomatous disease who lack p67 phox.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-3616R-A488)
Dodávateľ: BIOSS INC
Opis: NCF4 is a cytosolic oxidase associated protein. It is a component of the nicotinamide adenine dinucleotide phosphate oxidase, which mediates down regulation of NADPH oxidase through interactions with its SH3 domain. NCF4 is associated with p67 phox but is absent in patients with chronic granulomatous disease who lack p67 phox.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-9631R-A488)
Dodávateľ: BIOSS INC
Opis: 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.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-9631R-CY5)
Dodávateľ: BIOSS INC
Opis: 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.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-9631R-A750)
Dodávateľ: BIOSS INC
Opis: 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.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-9631R-CY7)
Dodávateľ: BIOSS INC
Opis: 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.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-3616R-CY3)
Dodávateľ: BIOSS INC
Opis: NCF4 is a cytosolic oxidase associated protein. It is a component of the nicotinamide adenine dinucleotide phosphate oxidase, which mediates down regulation of NADPH oxidase through interactions with its SH3 domain. NCF4 is associated with p67 phox but is absent in patients with chronic granulomatous disease who lack p67 phox.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-3497R-A647)
Dodávateľ: BIOSS INC
Opis: NCF4 is a cytosolic oxidase associated protein. It is a component of the nicotinamide adenine dinucleotide phosphate oxidase, which mediates down regulation of NADPH oxidase through interactions with its SH3 domain. NCF4 is associated with p67 phox but is absent in patients with chronic granulomatous disease who lack p67 phox.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-3497R-CY3)
Dodávateľ: BIOSS INC
Opis: NCF4 is a cytosolic oxidase associated protein. It is a component of the nicotinamide adenine dinucleotide phosphate oxidase, which mediates down regulation of NADPH oxidase through interactions with its SH3 domain. NCF4 is associated with p67 phox but is absent in patients with chronic granulomatous disease who lack p67 phox.
Merná jednotka: 1 * 100 µl


Dodávateľ: MP BIOMEDICALS
Opis: Lactulose is a synthetic, non-digestible sugar used in the treatment of chronic constipation and hepatic encephalopathy, a complication of liver disease. It is a disaccharide (double-sugar) formed from one molecule each of the simple sugars (monosaccharides) fructose and galactose.

Katalógové číslo: (BOSSBS-3616R-A555)
Dodávateľ: BIOSS INC
Opis: NCF4 is a cytosolic oxidase associated protein. It is a component of the nicotinamide adenine dinucleotide phosphate oxidase, which mediates down regulation of NADPH oxidase through interactions with its SH3 domain. NCF4 is associated with p67 phox but is absent in patients with chronic granulomatous disease who lack p67 phox.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-3616R-FITC)
Dodávateľ: BIOSS INC
Opis: NCF4 is a cytosolic oxidase associated protein. It is a component of the nicotinamide adenine dinucleotide phosphate oxidase, which mediates down regulation of NADPH oxidase through interactions with its SH3 domain. NCF4 is associated with p67 phox but is absent in patients with chronic granulomatous disease who lack p67 phox.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-9631R-A647)
Dodávateľ: BIOSS INC
Opis: 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.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-9631R-CY3)
Dodávateľ: BIOSS INC
Opis: 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.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (BOSSBS-9631R-A680)
Dodávateľ: BIOSS INC
Opis: 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.
Merná jednotka: 1 * 100 µl


Katalógové číslo: (PRSI29-625)
Dodávateľ: PROSCI
Opis: PKLR is a pyruvate kinase that catalyzes the production of phohsphoenolpyruvate from pyruvate and ATP. Defects in this enzyme, due to gene mutations or genetic variations, are the common cause of chronic hereditary nonspherocytic hemolytic anemia (CNSHA or HNSHA).The protein encoded by this gene is a pyruvate kinase that catalyzes the production of phohsphoenolpyruvate from pyruvate and ATP. Defects in this enzyme, due to gene mutations or genetic variations, are the common cause of chronic hereditary nonspherocytic hemolytic anemia (CNSHA or HNSHA). Alternatively spliced transcript variants encoding distinct isoforms have been described.
Merná jednotka: 1 * 100 µG


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