Name: Anti-MANBA Antibody (CAB15289)
Brand: Cell Biology Antibodies 5
SKU: CAB15289
Availability: OutOfStock

Antibody Name:	Anti-MANBA Antibody
Antibody SKU:	CAB15289
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Human, Mouse
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 18-300 of human MANBA (NP_005899.3).

Application:	WB IHC
Recommended Dilution:	WB 1:200 - 1:2000 IHC 1:50 - 1:200
Reactivity:	Human, Mouse
Positive Samples:	Mouse lung, Mouse testis

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 18-300 of human MANBA (NP_005899.3).
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:	AAEL SYSL RGNW SICN GNGS LELP GAVP GCVH SALF QQGL IQDS YYRF NDLN YRWV SLDN WTYS KEFK IPFE ISKW QKVN LILE GVDT VSKI LFNE VTIG ETDN MFNR YSFD ITNV VRDV NSIE LRFQ SAVL YAAQ QSKA HTRY QVPP DCPP LVQK GECH VNFV RKEQ CSFS WDWG PSFP TQGI WKDV RIEA YNIC HLNY FTFS PIYD KSAQ EWNL EIES TFDV VSSK PVGG QVIV AIPK LQTQ QTYS IELQ PGKR IVEL FVNI SKNI TVET WWPH GHGN QTG
Gene ID:	4126
Uniprot:	O00462
Cellular Location:	Lysosome
Calculated MW:	100kDa
Observed MW:	101kDa

Synonyms:	MANBA, MANB1
Background:	This gene encodes a member of the glycosyl hydrolase 2 family. The encoded protein localizes to the lysosome where it is the final exoglycosidase in the pathway for N-linked glycoprotein oligosaccharide catabolism. Mutations in this gene are associated with beta-mannosidosis, a lysosomal storage disease that has a wide spectrum of neurological involvement.

UniProt Protein Function:	MANBA: Exoglycosidase that cleaves the single beta-linked mannose residue from the non-reducing end of all N-linked glycoprotein oligosaccharides. Defects in MANBA are the cause of a mild disorder that affects peripheral and central nervous system myelin. Defects in MANBA are the cause of lysosomal beta- mannosidosis (LYSBMAN). Beta-mannosidosis is an autosomal recessive lysosomal storage disease of glycoprotein catabolism. Clinical features are heterogeneous with a wide range of symptoms and age of onset. The disease is associated with a range of neurological involvement, including various degrees of mental retardation in most of the cases, hearing loss and speech impairment, hypotonia, epilepsy and peripheral neuropathy. Affected individuals have a profound reduction in beta-mannosidase activity in plasma, fibroblasts and leukocytes. Belongs to the glycosyl hydrolase 2 family.
UniProt Protein Details:	Protein type:EC 3.2.1.25; Glycan Metabolism - other glycan degradation; Hydrolase Chromosomal Location of Human Ortholog: 4q24 Cellular Component: intracellular membrane-bound organelle; lysosomal lumen Molecular Function:beta-mannosidase activity Biological Process: carbohydrate metabolic process; oligosaccharide catabolic process Disease: Mannosidosis, Beta A, Lysosomal
NCBI Summary:	This gene encodes a member of the glycosyl hydrolase 2 family. The encoded protein localizes to the lysosome where it is the final exoglycosidase in the pathway for N-linked glycoprotein oligosaccharide catabolism. Mutations in this gene are associated with beta-mannosidosis, a lysosomal storage disease that has a wide spectrum of neurological involvement. [provided by RefSeq, Jul 2008]
UniProt Code:	O00462
NCBI GenInfo Identifier:	51338734
NCBI Gene ID:	4126
NCBI Accession:	O00462.3
UniProt Secondary Accession:	O00462,Q96BC3, Q9NYX9,
UniProt Related Accession:	O00462
Molecular Weight:	100,895 Da
NCBI Full Name:	Beta-mannosidase
NCBI Synonym Full Names:	mannosidase beta
NCBI Official Symbol:	MANBA
NCBI Official Synonym Symbols:	MANB1
NCBI Protein Information:	beta-mannosidase
UniProt Protein Name:	Beta-mannosidase
UniProt Synonym Protein Names:	Lysosomal beta A mannosidase; Mannanase; Mannase
Protein Family:	Beta-mannosidase
UniProt Gene Name:	MANBA
UniProt Entry Name:	MANBA_HUMAN

	Western blot analysis of extracts of various cell lines, using MANBA antibody (CAB15289) 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: 30s.
	Immunohistochemistry of paraffin-embedded human liver cancer using MANBA antibody (CAB15289) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human thyroid cancer using MANBA antibody (CAB15289) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human colon carcinoma using MANBA antibody (CAB15289) at dilution of 1:100 (40x lens).