Anti-SGSH 항체 (CAB8148)

Antibody Name:	SGSH Rabbit Polyclonal Antibody
Antibody SKU:	CAB8148
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 273-502 of human SGSH (NP_000190.1).

Application:	WB IHC
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	MCF7, 22Rv1, HepG2, Mouse liver, Mouse testis

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 273-502 of human SGSH (NP_000190.1).
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:	DNGI PFPS GRTN LYWP GTAE PLLV SSPE HPKR WGQV SEAY VSLL DLTP TILD WFSI PYPS YAIF GSKT IHLT GRSL LPAL EAEP LWAT VFGS QSHH EVTM SYPM RSVQ HRHF RLVH NLNF KMPF PIDQ DFYV SPTF QDLL NRTT AGQP TGWY KDLR HYYY RARW ELYD RSRD PHET QNLA TDPR FAQL LEML RDQL AKWQ WETH DPWV CAPD GVLE EKLS PQCQ PLHN EL
Gene ID:	6448
Uniprot:	P51688
Cellular Location:	Lysosome
Calculated MW:	56kDa
Observed MW:	63kDa

Synonyms:	SGSH, HSS, MPS3A, SFMD
Background:	This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.

UniProt Protein Function:	SGSH: Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A); also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival. Belongs to the sulfatase family.
UniProt Protein Details:	Protein type:Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; EC 3.10.1.1 Chromosomal Location of Human Ortholog: 17q25.3 Cellular Component: lysosomal lumen Molecular Function:N-sulfoglucosamine sulfohydrolase activity; metal ion binding; sulfuric ester hydrolase activity; catalytic activity Biological Process: proteoglycan metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; pathogenesis Disease: Mucopolysaccharidosis, Type Iiia
NCBI Summary:	This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined. [provided by RefSeq, Jul 2008]
UniProt Code:	P51688
NCBI GenInfo Identifier:	4506919
NCBI Gene ID:	6448
NCBI Accession:	NP_000190.1
UniProt Secondary Accession:	P51688,A8K5E2,
UniProt Related Accession:	P51688
Molecular Weight:	56,695 Da
NCBI Full Name:	N-sulphoglucosamine sulphohydrolase
NCBI Synonym Full Names:	N-sulfoglucosamine sulfohydrolase
NCBI Official Symbol:	SGSH
NCBI Official Synonym Symbols:	HSS; SFMD; MPS3A
NCBI Protein Information:	N-sulphoglucosamine sulphohydrolase; sulphamidase; heparan sulfate sulfatase; sulfoglucosamine sulfamidase; mucopolysaccharidosis type IIIA
UniProt Protein Name:	N-sulphoglucosamine sulphohydrolase
UniProt Synonym Protein Names:	Sulfoglucosamine sulfamidase; Sulphamidase
Protein Family:	N-sulphoglucosamine sulphohydrolase
UniProt Gene Name:	SGSH
UniProt Entry Name:	SPHM_HUMAN

	Western blot analysis of extracts of various cell lines, using SGSH antibody (CAB8148) 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: 10s.
	Immunohistochemistry of paraffin-embedded human liver using SGSH antibody (CAB8148) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human stomach using SGSH antibody (CAB8148) at dilution of 1:100 (40x lens).

Antibodies

SGSH Antibody (PACO12169)

Secondary Antibody

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