供货周期: | 现货 |
品牌: | 康朗生物 |
规格: | 0.1ml/0.2ml |
货号: | KL9631R |
CAS号: |
Quantity size:
0.2ml
Concentration:
1mg/ml 16号染色体开放阅读框57抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
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.16号染色体开放阅读框57抗体 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 16号染色体开放阅读框57抗体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.
16号染色体开放阅读框57抗体Also known as:
Chromosome 16 open reading frame 57; CP057_HUMAN; FLJ13154; UPF0406 protein C16orf57.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
●
Reacts with: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, .
●
Immunogen: KLH conjugated synthetic peptide derived from human C16orf57.
●
16号染色体开放阅读框57抗体Predicted Molecular Weight: 30kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
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