{"code":"SASDW74","status":"Published","type_of_curve":"SEC-SAS","angular_unit":"1/nm","project":{"title":"The CRISPR-Cas9 knockout DDC SH-SY5Y in vitro model for AADC deficiency provides insight into the pathogenicity of R347Q and L353P variants: a cross-sectional structural and functional analysis.","publication":{"title":"The CRISPR-Cas9 knockout DDC SH-SY5Y in vitro model for AADC deficiency provides insight into the pathogenicity of R347Q and L353P variants: a cross-sectional structural and functional analysis.","author_list":"Carmona-Carmona CA, Bisello G, Franchini R, Lunardi G, Galavotti R, Perduca M, Ribeiro RP, Belviso BD, Giorgetti A, Caliandro R, Lievens PM, Bertoldi M","journal":"FEBS J","doi":"10.1111/febs.70120","pmid":"40318155","published_date":"2025 May 3"},"status":"released","submitted_date":"2024-12-20","released_date":"2025-04-02"},"pddf_data":"https://www.sasbdb.org/media/p_of_R_files/SASDW74.out","intensities_data":"https://www.sasbdb.org/media/intensities_files/SASDW74.dat","intensities_log_plot":"https://www.sasbdb.org/media/intensities_files/scattering_plots/SASDW74_dat_img.png","intensities_kratky_plot":"https://www.sasbdb.org/media/intensities_files/scattering_plots/SASDW74_kratky_img.png","pddf_plot":"https://www.sasbdb.org/media/p_of_R_files/pofr_images/SASDW74_pofr_img.png","intensities_guinier_plot":"https://www.sasbdb.org/media/intensities_files/scattering_plots/SASDW74_guinier_img.png","sascif_data":"https://www.sasbdb.org/media/sascif/sascif_files/SASDW74.sascif","experiment":{"instrument":{"detector":{"type":null,"name":"Eiger 4M","resolution":75.0},"name":"Diamond Light Source","city":"Didcot","country":"UK","beamline_name":"B21","beam_geometry":null,"type_of_source":"X-ray synchrotron","point_source":null,"line_collimation":null,"sample_path_length":null,"line_collimation_slitlength":null,"line_collimation_integrationwidth":null,"xray_energy":null,"beam_profile_ah":null,"beam_profile_al":null},"sample":{"molecule":[{"long_name":"Aromatic-L-amino-acid decarboxylase (L353P)","short_name":"AADC","sequence":"MNASEFRRRGKEMVDYMANYMEGIEGRQVYPDVEPGYLRPLIPAAAPQEPDTFEDIINDVEKIIMPGVTHWHSPYFFAYFPTASSYPAMLADMLCGAIGCIGFSWAASPACTELETVMMDWLGKMLELPKAFLNEKAGEGGGVIQGSASEATLVALLAARTKVIHRLQAASPELTQAAIMEKLVAYSSDQAHSSVERAGLIGGVKLKAIPSDGNFAMRASALQEALERDKAAGLIPFFMVATLGTTTCCSFDNLLEVGPICNKEDIWLHVDAAYAGSAFICPEFRHLLNGVEFADSFNFNPHKWLLVNFDCSAMWVKKRTDLTGAFRLDPTYLKHSHQDSGLITDYRHWQIPPGRRFRSLKMWFVFRMYGVKGLQAYIRKHVQLSHEFESLVRQDPRFEICVEVILGLVCFRLKGSNKVNEALLQRINSAKKIHLVPCHLRDKFVLRFAICSRTVESAHVQRAWEHIKELAADVLRAE","organism":"Homo sapiens","uniprot_code":"P20711","uniprot_range_first":1,"uniprot_range_last":478,"oligomerization":"dimer","molecular_type":"protein","uniprot_sequence":"MNASEFRRRGKEMVDYMANYMEGIEGRQVYPDVEPGYLRPLIPAAAPQEPDTFEDIINDV\nEKIIMPGVTHWHSPYFFAYFPTASSYPAMLADMLCGAIGCIGFSWAASPACTELETVMMD\nWLGKMLELPKAFLNEKAGEGGGVIQGSASEATLVALLAARTKVIHRLQAASPELTQAAIM\nEKLVAYSSDQAHSSVERAGLIGGVKLKAIPSDGNFAMRASALQEALERDKAAGLIPFFMV\nATLGTTTCCSFDNLLEVGPICNKEDIWLHVDAAYAGSAFICPEFRHLLNGVEFADSFNFN\nPHKWLLVNFDCSAMWVKKRTDLTGAFRLDPTYLKHSHQDSGLITDYRHWQIPLGRRFRSL\nKMWFVFRMYGVKGLQAYIRKHVQLSHEFESLVRQDPRFEICVEVILGLVCFRLKGSNKVN\nEALLQRINSAKKIHLVPCHLRDKFVLRFAICSRTVESAHVQRAWEHIKELAADVLRAERE","mw":53.624,"total_mw":107.248,"number_molecules":2,"complex_state":false,"deuteration":null,"molecule_source":"biological","molecule_description":""}],"buffer":{"name":"50 mM HEPES","concentration_unit":null,"comment":null,"additive":null,"concentration":null,"pka":null,"ph":7.4,"deuteration":null},"purity_method":null,"name":"Aromatic-L-amino-acid decarboxylase L353P bound to pyridoxal 5'-phosphate (PLP)","ext_coefficient":null,"contrast":null,"specific_vol":null,"dry_vol":null,"absorbption":null,"deuteration":null,"mixture":null},"contributor":[{"affiliation":[{"short_name":null,"address":null,"full_name":"National Research Council","webpage":null}],"contributor_name":"Benny Danilo","contributor_surname":"Belviso","orcid":"https://orcid.org/0000-0003-2880-1064"}],"concentration_method":null,"concentration_unit":null,"date":"2021-07-07","storage_temperature":20.0,"cell_temperature":20.0,"exposure_time":3.0,"number_of_frames":620,"wavelength":0.1,"sample_detector_distance":3.7,"concentration_min":null,"concentration_max":4.0,"sample_volume":50.0,"flow_rate":0.16,"s_min":0.045,"s_max":3.4,"total_exposure_time":null,"seccolumn":19},"fits":[{"models":[{"model_plot":"https://www.sasbdb.org/media/pdb_file/images/SASDW74_fit1_model1_img.png","software":"DAMMIN","pdb_link":[],"model_title":null,"type_of_model":"dummy","software_version":"3.0.2","model_data":"https://www.sasbdb.org/media/pdb_file/SASDW74_fit1_model1.pdb","model_mw":86.19,"bead_radius":2.5,"log":"https://www.sasbdb.org/media/log_files/dammin_P1_refinement.log","symmetry":null,"comment":null,"user":930}],"fit_unit":"1/A","fit_plot":"https://www.sasbdb.org/media/fitting_files/scattering_plots/SASDW74_fit1_fixed_fit_img.png","software":"DAMMIN","chi_square_value":1.288,"p_value":0.0007,"fit_residual_plot":"SASDW74_fit1_fitresiduals_img.png","fit_data":"https://www.sasbdb.org/media/fitting_files/SASDW74_fit1.fir","fit_log":"https://www.sasbdb.org/media/fitting_files/log_files/dammin_P1_refinement.log","software_version":null,"description":""}],"estimated_volume_method":null,"pddf_software":"ATSAS GNOM","pddf_software_version":"5.0","i0_calibration_standard":null,"description":"Pyridoxal 5'-phosphate: https://www.ebi.ac.uk/chebi/searchId.do?chebiId=CHEBI:18405. CAUTION: Data used to calculate p(r) have been significantly truncated at both low- and high-s.","experiment_description":"Synchrotron SAXS data from solutions of aromatic-L-amino-acid decarboxylase (L353P) bound to pyridoxal 5'-phosphate in 50 mM HEPES, pH 7.4 were collected on the B21 beam line at the Diamond Light Source (Didcot, UK) using a Eiger 4M detector at a sample-detector distance of 3.7 m and at a wavelength of λ = 0.1 nm (I(s) vs s, where s = 4πsinθ/λ, and 2θ is the scattering angle). In-line size-exclusion chromatography (SEC) SAS was employed. The SEC parameters were as follows: A 50.00 μl sample at 4 mg/ml was injected at a 0.16 ml/min flow rate onto a Shodex KW403-4F column at 20°C. 620 successive 3 second frames were collected. The data were normalized to the intensity of the transmitted beam and radially averaged; the scattering of the solvent-blank was subtracted.","tags":[],"intensity_unit":"arbitrary","experimental_mw":109.0,"experimental_mw_error":null,"guinier_i0_mw":null,"guinier_i0_mw_error":null,"porod_mw":111.356,"porod_mw_error":null,"pddf_i0":0.1592,"pddf_i0_error":0.0002,"guinier_i0":0.167542,"guinier_i0_error":null,"pddf_rg":2.91,"pddf_rg_error":null,"guinier_rg":3.088,"guinier_rg_error":0.002,"pddf_dmax":8.79,"pddf_dmax_error":null,"porod_volume":151.97,"porod_volume_error":null,"estimated_volume":null,"estimated_volume_error":null,"guinier_point_first":19,"guinier_point_last":275,"pddf_point_first":null,"pddf_point_last":null,"i0_calibration_standard_data":null,"intensities_log_log_plot":"SASDW74_datloglog_img.png","symmetry":null,"last_modified":"2025-05-08T08:55:23.578994+02:00","bragg_peak":[]}