HPS1 polyclonal, anti-human, mouse, rat
€388.00
In stock
SKU
BS8302
Background:
Hermansky-Pudlak syndrome (HPS) is a rare, genetically heterogeneous, autosomal recessive disorder. It is characterized by oculocutaneous albinism, lysosomal storage defects and prolonged bleeding due to platelet storage pool deficiency. There are 10 HPS genes encoding HPS proteins that all interact within three distinct ubiquitously expressed protein complexes or biogenesis of lysosome-related organelle complexes. Defects in these genes cause HPS. HSP-1 is a component of multiple cytoplasmic organelles and is important for their normal development and function. It plays a role in intracellular protein sorting.
Alternative Name:
Hermansky-Pudlak syndrome 1 protein, HPS1, HPS
Application Dilution: WB: 1:500~1:2000, IHC/IF: 1:50~1:200
Specificity: HPS1 polyclonal antibody detects endogenous levels of HPS1 protein.
Immunogen:
Recombinant full length Human HPS1.
MW: ~ 79 kDa
Swis Prot.: Q92902
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
Hermansky-Pudlak syndrome (HPS) is a rare, genetically heterogeneous, autosomal recessive disorder. It is characterized by oculocutaneous albinism, lysosomal storage defects and prolonged bleeding due to platelet storage pool deficiency. There are 10 HPS genes encoding HPS proteins that all interact within three distinct ubiquitously expressed protein complexes or biogenesis of lysosome-related organelle complexes. Defects in these genes cause HPS. HSP-1 is a component of multiple cytoplasmic organelles and is important for their normal development and function. It plays a role in intracellular protein sorting.
Alternative Name:
Hermansky-Pudlak syndrome 1 protein, HPS1, HPS
Application Dilution: WB: 1:500~1:2000, IHC/IF: 1:50~1:200
Specificity: HPS1 polyclonal antibody detects endogenous levels of HPS1 protein.
Immunogen:
Recombinant full length Human HPS1.
MW: ~ 79 kDa
Swis Prot.: Q92902
Purification & Purity:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Format:
1mg/ml in PBS with 0.1% Sodium Azide, 50% Glycerol.
Storage:
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
For research use only, not for use in diagnostic procedure.
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