Anti-HPS4 항체 (CAB16157)

Antibody Name:	Anti-HPS4 Antibody
Antibody SKU:	CAB16157
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	A synthetic peptide corresponding to a sequence within amino acids 550-650 of human HPS4 (NP_690054.1).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	U-87MG, SGC-7901, LO2, Jurkat, Mouse liver

Immunogen:	A synthetic peptide corresponding to a sequence within amino acids 550-650 of human HPS4 (NP_690054.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	LLAE EPLL GDSA AIEE VYHS SLAS LNGL EVHL KETL PRDE AAST SSTY NFTH YDRI QSLL MANL PQVA TPQD RRFL QAVS LMHS EFAQ LPAL YEMT VRNA S
Gene ID:	89781
Uniprot:	Q9NQG7
Cellular Location:
Calculated MW:	58kDa/76kDa/78kDa
Observed MW:	115kDa

Synonyms:	HPS4, BLOC3S2, LE
Background:	This gene encodes a protein component of biogenesis of lysosome-related organelles complexes (BLOC). BLOC complexes are important for the formation of endosomal-lysosomal organelles such as melanosomes and platelet dense granules. Mutations in this gene result in subtype 4 of Hermansky-Pudlak syndrome, a form of albinism. Alternative splicing results in multiple transcript variants.

UniProt Protein Function:	HPS4: May function in the pathway of organelle biogenesis. Defects in HPS4 are the cause of Hermansky-Pudlak syndrome type 4 (HPS4). Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. 4 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Chromosomal Location of Human Ortholog: 22cen-q12.3 Cellular Component: platelet dense granule; membrane; lysosome; cytoplasm; melanosome Molecular Function:protein dimerization activity; protein binding; protein homodimerization activity Biological Process: positive regulation of eye pigmentation; protein stabilization; lysosome organization and biogenesis; hemostasis; melanocyte differentiation; blood coagulation; protein targeting Disease: Hermansky-pudlak Syndrome 4
NCBI Summary:	This gene encodes a protein component of biogenesis of lysosome-related organelles complexes (BLOC). BLOC complexes are important for the formation of endosomal-lysosomal organelles such as melanosomes and platelet dense granules. Mutations in this gene result in subtype 4 of Hermansky-Pudlak syndrome, a form of albinism. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2012]
UniProt Code:	Q9NQG7
NCBI GenInfo Identifier:	21362617
NCBI Gene ID:	89781
NCBI Accession:	Q9NQG7.2
UniProt Secondary Accession:	Q9NQG7,Q5H8V6, Q96LX6, Q9BY93, Q9UH37, Q9UH38, B1AHQ4
UniProt Related Accession:	Q9NQG7
Molecular Weight:	78,648 Da
NCBI Full Name:	Hermansky-Pudlak syndrome 4 protein
NCBI Synonym Full Names:	Hermansky-Pudlak syndrome 4
NCBI Official Symbol:	HPS4
NCBI Official Synonym Symbols:	LE
NCBI Protein Information:	Hermansky-Pudlak syndrome 4 protein; light-ear protein homolog
UniProt Protein Name:	Hermansky-Pudlak syndrome 4 protein
UniProt Synonym Protein Names:	Light-ear protein homolog
Protein Family:	Hermansky-Pudlak syndrome 4 protein
UniProt Gene Name:	HPS4
UniProt Entry Name:	HPS4_HUMAN

	Western blot analysis of extracts of various cell lines, using HPS4 antibody (CAB16157) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.
	Immunofluorescence analysis of C6 cells using HPS4 antibody (CAB16157) at dilution of 1:100. Blue: DAPI for nuclear staining.
	Immunofluorescence analysis of L929 cells using HPS4 antibody (CAB16157) at dilution of 1:100. Blue: DAPI for nuclear staining.

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)