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Image Unavailable-BOSSBS-9631R-CY5.5

Anti-USB1 Rabbit Polyclonal Antibody (Cy5.5®)

Catalog Number: (BOSSBS-9631R-CY5.5)
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.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-9631R-A555

Anti-USB1 Rabbit Polyclonal Antibody (Alexa Fluor® 555)

Catalog Number: (BOSSBS-9631R-A555)
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.
UOM: 1 * 100 µl

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Image Unavailable-BOSSBS-9631R-A750

Anti-C16orf57 Rabbit Polyclonal Antibody (Alexa Fluor® 750)

Catalog Number: (BOSSBS-9631R-A750)
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.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-9631R-CY7

Anti-USB1 Rabbit Polyclonal Antibody (Cy7®)

Catalog Number: (BOSSBS-9631R-CY7)
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.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-9631R-A488

Anti-USB1 Rabbit Polyclonal Antibody (Alexa Fluor® 488)

Catalog Number: (BOSSBS-9631R-A488)
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.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-9631R-CY5

Anti-USB1 Rabbit Polyclonal Antibody (Cy5®)

Catalog Number: (BOSSBS-9631R-CY5)
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.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-9631R

Anti-C16orf57 Rabbit Polyclonal Antibody

Catalog Number: (BOSSBS-9631R)
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.
UOM: 1 * 100 µl
Product Image-765-0090

Thomson e / m tube

Catalog Number: (765-0090)
Supplier: 3B Scientific
Description: Evacuated glass flask containing i.a. electron gun with directly heated tungsten wire cathode and a cylindrical, collimating anode. The generated beam is allowed to stroke along a semi-transparent mica disc in the center of the tube. Luminescent coating on the front of the disc makes the beam appear. Two of the edges of the back are provided with graduated scales.
UOM: 1 * 1 items

Market Source Item This is a MarketSource item. Additional charges may apply

Product Image-216-2259

VWR®, Instrument trays with lid

Supplier: VWR Collection
Description: These instrument trays are made from rust-proof, electrolytically polished stainless steel.

Product Image-462-0574

VWR®, Stainless Steel Base Trays

Supplier: VWR Collection
Description: Stainless steel base tray, for VWB2 12

Product Image-462-0500

VWR®, Trays for VLSB Shaking Baths

Supplier: VWR Collection
Description: Shaking bath, Stainless steel base tray (converts bath to unstirred bath), for VLSB18/VKB18

Product Image-216-0050

Instrument trays, with lid

Supplier: BOCHEM
Description: Stainless steel, 18/10.

Product Image-83650.290

Water, HiPerSolv CHROMANORM®, super gradient grade for HPLC, suitable for UPLC/UHPLC instruments

Supplier: VWR Chemicals
Description: Water, HiPerSolv CHROMANORM®, super gradient grade for HPLC, suitable for UPLC/UHPLC instruments

SDS Certificates Environmentally Preferable

Product Image-462-0577

VWR®, Spare Polycarbonate Base Trays

Supplier: VWR Collection
Description: Polycarbonate base tray, for VWB2 2, Polycarbonate base tray, for VWB2 2

Product Image-83645.290

Water, HiPerSolv CHROMANORM® for LC-MS, suitable for UPLC/UHPLC instruments

Supplier: VWR Chemicals
Description: Water, HiPerSolv CHROMANORM® for LC-MS, suitable for UPLC/UHPLC instruments

SDS Certificates Environmentally Preferable Sale

Product Image-BOCH8486

Instrument trays

Supplier: BOCHEM
Description: Made of 18/10 steel.

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Stock for this item is limited, but may be available in a warehouse close to you. Please make sure that you are logged in to the site so that available stock can be displayed. If the call is still displayed and you need assistance, please call us on 0800 22 33 44
Additional Documentation may be needed to purchase this item. A VWR representative will contact you if needed.
Additional Documentation may be needed to purchase this item. A VWR representative will contact you if needed.
This product has been blocked by your organisation. Please contact your purchasing department for more information.
The original product is no longer available. The replacement shown is available.
Product(s) marked with this symbol are discontinued - sold till end of stock. Alternatives may be available by searching with the VWR Catalogue Number listed above. If you need further assistance, please call VWR Customer Service on 0800 22 33 44.
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