Anti-COL4A3BP 항체 (CAB15376)

Antibody Name:	Anti-COL4A3BP Antibody
Antibody SKU:	CAB15376
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 369-598 of human COL4A3BP (NP_112729.1).

Application:	WB IF
Recommended Dilution:	WB 1:200 - 1:2000 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	LO2, A-549, HeLa, Mouse thymus, Mouse brain, Mouse heart

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 369-598 of human COL4A3BP (NP_112729.1).
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:	QKVE EMVQ NHMT YSLQ DVGG DANW QLVV EEGE MKVY RREV EENG IVLD PLKA THAV KGVT GHEV CNYF WNVD VRND WETT IENF HVVE TLAD NAII IYQT HKRV WPAS QRDV LYLS VIRK IPAL TEND PETW IVCN FSVD HDSA PLNN RCVR AKIN VAMI CQTL VSPP EGNQ EISR DNIL CKIT YVAN VNPG GWAP ASVL RAVA KREY PKFL KRFT SYVQ EKTA GKPI LF
Gene ID:	10087
Uniprot:	Q9Y5P4
Cellular Location:	Cytoplasm, Endoplasmic reticulum, Golgi apparatus
Calculated MW:	68kDa/70kDa/83kDa
Observed MW:	71kDa

Synonyms:

COL4A3BP, CERT, CERTL, GPBP, MRD34, STARD11

Background:

This gene encodes a kinase that specifically phosphorylates the N-terminal region of the non-collagenous domain of the alpha 3 chain of type IV collagen, known as the Goodpasture antigen. Goodpasture disease is the result of an autoimmune response directed at this antigen. One isoform of this protein is also involved in ceramide intracellular transport. Three transcript variants encoding different isoforms have been found for this gene.

UniProt Protein Function:	COL4A3BP: a protein with a lipid-binding domain that mediates intracellular trafficking of ceramide in a non-vesicular manner. Implicated in human autoimmune diseases via its association with the Goodpasture antigen. Phosphorylation on Ser-132 decreases the affinity toward phosphatidylinositol 4-phosphate at Golgi membranes and reduces ceramide transfer activity. GPBP has been reported to have Ser/Thr protein kinase activity towards the Goodpasture (GP) antigen, but lacks a conventional serine/threonine kinase domain. Two human isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Kinase, protein; Protein kinase, Ser/Thr (non-receptor); EC 2.7.11.9; Lipid-binding; Protein kinase, atypical; ATYPICAL group; COL4A3BP family Chromosomal Location of Human Ortholog: 5q13.3 Cellular Component: nucleoplasm; Golgi apparatus; endoplasmic reticulum membrane; mitochondrion; perinuclear region of cytoplasm; cytosol Molecular Function:protein binding; protein kinase activity Biological Process: heart morphogenesis; endoplasmic reticulum organization and biogenesis; cell proliferation; sphingolipid metabolic process; muscle contraction; sphingolipid biosynthetic process; in utero embryonic development; cell morphogenesis; lipid homeostasis; immune response; signal transduction; ceramide metabolic process; protein amino acid phosphorylation Disease: Mental Retardation, Autosomal Dominant 34
NCBI Summary:	This gene encodes a kinase that specifically phosphorylates the N-terminal region of the non-collagenous domain of the alpha 3 chain of type IV collagen, known as the Goodpasture antigen. Goodpasture disease is the result of an autoimmune response directed at this antigen. One isoform of this protein is also involved in ceramide intracellular transport. Three transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
UniProt Code:	Q9Y5P4
NCBI GenInfo Identifier:	20978413
NCBI Gene ID:	10087
NCBI Accession:	Q9Y5P4.1
UniProt Secondary Accession:	Q9Y5P4,Q53YV1, Q53YV2, Q96Q85, Q96Q88, Q9H2S7, Q9H2S8 A8K7S2, B3KUB7,
UniProt Related Accession:	Q9Y5P4
Molecular Weight:	83,708 Da
NCBI Full Name:	Collagen type IV alpha-3-binding protein
NCBI Synonym Full Names:	collagen, type IV, alpha 3 (Goodpasture antigen) binding protein
NCBI Official Symbol:	COL4A3BP
NCBI Official Synonym Symbols:	CERT; GPBP; CERTL; STARD11
NCBI Protein Information:	collagen type IV alpha-3-binding protein; hCERT; ceramide transporter; ceramide transfer protein; lipid-transfer protein CERTL; StAR-related lipid transfer (START) domain containing 11
UniProt Protein Name:	Collagen type IV alpha-3-binding protein
UniProt Synonym Protein Names:	Ceramide transfer protein; hCERT; Goodpasture antigen-binding protein; GPBP; START domain-containing protein 11; StARD11; StAR-related lipid transfer protein 11
Protein Family:	Collagen type IV alpha-3-binding protein
UniProt Gene Name:	COL4A3BP
UniProt Entry Name:	C43BP_HUMAN

	Western blot analysis of extracts of various cell lines, using COL4A3BP antibody (CAB15376) 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: 15s.
	Immunofluorescence analysis of L929 cells using COL4A3BP antibody (CAB15376) at dilution of 1:100. Blue: DAPI for nuclear staining.

Antibodies	ELISA Kits
COL4A3BP Antibody (PACO08512)	Human COL4A3BP / Collagen type IV alpha 3 binding protein ELISA Kit

Secondary Antibody

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